Sensorineural Hearing Loss in Children: Clinical Guidelines

List of Abbreviations

ASSR (auditory steady-state response)

GJB2 (gap junction protein beta 2 – gene encoding connexin 26)

HMT – high-tech medical care

WHO – World Health Organization

OSPL90 – output sound pressure level for a 90 dB input sound pressure level

Hz – Hertz

dB – decibel

dB nHL – decibel normal hearing level

IPRA – individual rehabilitation or habilitation program

CI – cochlear implant

ABR – auditory brainstem responses

CT (MSCT) – computed tomography (multi-slice computed tomography)

ICD-10 – International Classification of Diseases 10th Revision

ICF – International Classification of Functioning, Disability and Health

CM – cochlear microphonic

MRI – magnetic resonance imaging

MSE – medical and social expertise

OAE – otoacoustic emission

PCR – polymerase chain reaction

HA – hearing aid

ABI – auditory brainstem implant

SNHL – sensorineural hearing loss

ATD – assistive technology devices

CMVI – cytomegalovirus infection

CNS – central nervous system

Terms and Definitions

Habilitation – a system of medical, psychological, and pedagogical measures aimed at developing abilities for everyday, social, professional, and other activities that were previously absent in a hard-of-hearing or deaf child. Habilitation is aimed at eliminating or compensating as fully as possible for activity limitations for the purpose of social adaptation.

Pre-lingual hearing loss – hearing loss acquired before the acquisition of speech.

Bilateral (binaural) cochlear implantation – a set of audiological, deaf education, psychological, and surgical procedures for the placement of two cochlear implant systems in both ears.

Auditory neuropathy spectrum disorder (auditory neuropathy) – polymorphic changes reflecting a pathological condition of the auditory system, including sensory elements (inner hair cells), synapses, the auditory nerve, and structural elements of the brainstem, in which otoacoustic emissions (OAE) and/or cochlear microphonics (CM) are recorded, while auditory brainstem responses (ABR) are absent or significantly altered.

Individual rehabilitation or habilitation program (IPRA) – a set of rehabilitation measures optimal for an individual with a disability, including specific types, forms, scopes, timeframes, and procedures for implementing medical, vocational, and other rehabilitation measures aimed at restoring or compensating for impaired bodily functions and developing, restoring, or compensating for the individual's ability to perform specific types of activities.

Auditory brainstem responses (ABR) – responses of the auditory pathway structures to acoustic stimulation, recorded via electrodes fixed on the head within a time interval corresponding to the passage of acoustic information along the auditory nerve and brainstem. The study results are displayed as a complex of positive peaks (waves I–VII), of which wave V is the most stable.

Cochlear implantation (CI) – a method of (re)habilitation for patients with deafness using an electrical stimulation system of the auditory nerve placed in the cochlea, consisting of 3 stages: 1) patient selection; 2) surgery to insert the electrode array into the cochlea; 3) postoperative auditory-verbal (re)habilitation. The term "cochlear implantation" may also refer exclusively to the surgical procedure of placing the internal component of the cochlear implant system.

Cochlear implant (cochlear implant system, CI) – an electronic device that enables people with bilateral deafness or profound hearing loss to hear environmental sounds and speech. A CI consists of an internal part implanted in the cochlea of the temporal bone that stimulates the auditory nerve (including a receiver, electrode array, and grounding electrode) and an external part located on the patient's ear/head (including a speech processor, microphone, transmitter, and power supply). A CI transmits sounds and speech to the deaf person's auditory system via electrical stimulation of the auditory nerve.

Medical speech therapist / defectologist (speech therapist) – a specialist who works with children having speech disorders. Their tasks include restoring/developing speech skills and abilities, correcting speech defects, reducing the risk of progressive speech disorders, and speech adaptation in a new or unfamiliar communicative environment.

Otoacoustic emission (OAE) – low-intensity acoustic oscillations generated by the outer hair cells of the organ of Corti, which can be recorded by an acoustic probe in the external auditory canal. OAE evoked by acoustic stimulation is not recorded if hearing thresholds exceed 25–30 dB nHL (or 40–50 dB nHL for TPOAE test with 70/70 dB stimuli) in the frequency range from 1 kHz to 4–5 kHz or up to 8–10 kHz (depending on the type of OAE).

Late-deafened children – patients with post-lingual hearing loss (acquired after the acquisition of speech).

Post-lingual hearing loss – hearing loss acquired after the acquisition of speech.

Medical psychologist / educational psychologist – a specialist whose tasks include participating in the rehabilitation process of children with hearing impairment to ensure an individualized approach to the patient based on psychological diagnosis of their personality traits; prevention and overcoming of psychopathological deviations in the patient's personal sphere. Proficient in methods of psychodiagnosis, psychocorrection, psychological counseling, and psychoprophylaxis. In educational institutions, psychodiagnosis of a child's personality traits, psychological counseling, psychocorrection, and psychoprophylaxis are performed by an educational psychologist.

Early-deafened children – patients with pre-lingual hearing loss.

Rehabilitation of individuals with disabilities – a system and process of full or partial restoration of the abilities of individuals with disabilities for everyday, social, professional, and other activities. Habilitation of individuals with disabilities – a system and process of developing abilities for everyday, social, professional, and other activities that were previously absent in individuals with disabilities.

Rehabilitation diagnosis – a list of the patient's problems formulated in ICF categories and reflecting all relevant aspects of the patient's functioning, intended to serve as a basis for formulating the goal, objectives, and strategy of rehabilitation. The rehabilitation diagnosis includes only those identified problems of the patient that affect or reflect their functioning.

Speech processor (sound speech processor, audio processor, hearing processor) – the external part of the cochlear implant system designed for the perception of acoustic information, performing processing, coding, and subsequent transmission of the digital signal to the internal part of the cochlear implant system.

Family-centered approach to the (re)habilitation of a child with hearing impairment – an approach based on the maximum involvement of the family in the child care program.

Sensorineural hearing loss (SNHL) – hearing impairment caused by damage to the sound-perceiving structures of the auditory system, from the auditory receptors of the cochlea (hair cells) to the cortical centers.

Remote microphone system, FM system – an assistive technology device consisting of a receiver and a transmitter that provides direct transmission of the speaker's (teacher's) voice to the hearing aid or speech processor while improving the signal-to-noise ratio.

Hearing aid (HA) – a device that amplifies sounds and speech, enabling children with hearing loss to perceive them.

Auditory-verbal (re)habilitation – a system of medical, psychological, and pedagogical measures aimed at adapting a child with hearing impairments to life through the compensation of hearing deficits.

Auditory steady-state responses (ASSR) – auditory evoked potentials elicited in response to a continuous tonal stimulus modulated in amplitude and frequency.

Auditory brainstem implantation – a method of (re)habilitation for deaf children and adults using electrical stimulation of the cochlear nuclei of the brainstem, consisting of 3 stages: 1) patient selection for surgery; 2) surgical placement of the electrode carrier into the cochlear nuclei of the brainstem; 3) postoperative auditory-verbal (re)habilitation. Additionally, the term "auditory brainstem implantation" may refer exclusively to the surgical stage of internal auditory brainstem implant placement.

Auditory brainstem implant (ABI) – an electronic device that enables patients with bilateral deafness due to damage or aplasia of the auditory nerve, or anomaly or complete ossification of the cochlea, to perceive environmental sounds and speech. The ABI is placed in and stimulates the cochlear nuclei of the brainstem (proximal to the auditory nerve). The external part of the ABI is identical to a CI, while the internal part differs in the shape of the electrode carrier.

Surdoacoustic specialist – (in accordance with Order No. 205n dated May 2, 2023, "On Approval of the Nomenclature of Positions of Medical and Pharmaceutical Workers") a specialist with secondary vocational non-medical education - a specialist in the field of hearing aids and prosthetics (surdoacoustic technician).

Teacher of the deaf / deaf-educator – a specialist who works with children with hearing impairments following hearing aid fitting and/or cochlear implantation. Their tasks include: developing spoken language, teaching communication via conversational speech on an audio-visual and auditory basis; preventing the regression of speech in children who lost their hearing at an age when speech had already been established.

Assistive technology devices for rehabilitation – rehabilitation devices containing technical solutions, including specialized ones, used to compensate for or eliminate persistent or reversible activity limitations in individuals caused by health disorders, including persons with disabilities (GOST R 51632-2021)

Pure-tone audiometry – an instrumental method for assessing a child's hearing status, evaluating hearing thresholds for sounds of various frequencies via air and bone conduction.

Tono-verbal dissociation – a discrepancy between speech intelligibility and pure-tone hearing thresholds in a child (intelligibility is lower than expected based on the audiogram). It is characteristic of some children with auditory neuropathy, as well as lesions of the central auditory system.

Hearing loss – a decrease in hearing of varying degrees caused by damage to any parts of the sound-conducting and/or sound-perceiving divisions of the auditory system, ranging from the structures of the external ear to the cortical representation in the temporal lobe of the cerebral cortex.

Universal newborn hearing screening – hearing screening for all newborns in maternity hospitals and outpatient clinics (in the Russian Federation – using the objective method of otoacoustic emissions recording at stage 1 and auditory brainstem response (ABR) recording at stage 2) for the purpose of identifying children with suspected hearing impairment and referring them for diagnostic evaluation at a hearing center/office. A prerequisite for early diagnosis of hearing impairment in a child and timely rehabilitation.

Teacher-defectologist - (in accordance with Order of the Ministry of Health of the Russian Federation No. 178n dated April 9, 2015, "On Approval of the Procedure for Providing Medical Care to the Population in the Profile 'Surdo-Otorhinolaryngology'") – a pedagogical specialist involved in the education, upbringing, and development of children with various developmental disorders. The terms "Specialist in Special Education" and "Remedial Educator" are used as synonyms. Depending on the training profile, the following are distinguished: teacher-educator of the deaf (surdo-educator), teacher-typhlopedagogue, teacher-oligophrenopedagogue, speech-language pathologist. Children with hearing impairments are managed by a teacher-educator of the deaf (surdo-educator). The terms teacher-educator of the deaf (surdo-educator) and surdo-educator are used as synonyms in the document.

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Sensorineural hearing loss (SNHL) – a form of hearing reduction (up to complete loss) in which any of the parts of the sound-perceiving division of the auditory analyzer are damaged, ranging from the immediate sensory apparatus of the cochlea to the neural structures [1].

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

The main causes of SNHL in children can be divided into two large groups: hereditary (genetically determined, familial) and non-hereditary (exogenous). Regarding the time of onset, hearing impairments can be congenital (the factor affects the organism of the embryo, fetus, and newborn) and acquired.

Genotype alterations are the cause of hearing impairment in 70% of children with congenital and prelingual hearing loss, with 70–85% of cases being non-syndromic forms. More than 100 genes encode membrane, regulatory, and structural proteins of the inner ear. Mutations in these genes lead to dysfunction of the organ of Corti and, consequently, to peripheral hearing loss [2,3,107].

Mutations in the GJB2 gene are the most common. The 35delG mutation is the most prevalent in the Central and North-Western regions of Russia, with a heterozygous carrier frequency in the healthy population of 2–6%. The prevalence of this form of congenital hearing loss in the Russian Federation currently exceeds 50% among all children with permanent bilateral hearing impairment. The GJB2 gene encodes the synthesis of connexin 26 protein, which is a structural subunit of connexin channels responsible for K+ ion homeostasis in the cochlea. Recessive mutations of this gene result in congenital bilateral non-syndromic severe sensorineural hearing loss and deafness. Previously, such children constituted a group of unclear etiology, since the majority of them had no burdened family history and lacked other risk factors for hearing loss [4].

Syndromes associated with congenital SNHL primarily include Pendred syndrome (SLC26A4 gene), Usher syndrome type IIA (USH2A gene), branchio-oto-renal syndrome (EYA1 gene), Waardenburg syndrome, and others.

Congenital cytomegalovirus infection (cCMV) is currently considered the most frequent non-hereditary cause of congenital SNHL, detected in 1% of newborns in the general population and 25% of premature infants. In 10% of cases of congenital cCMV, non-specific symptoms develop, and sensorineural hearing loss is detected in half of these children. However, 90% of congenital cCMV cases are asymptomatic, and the incidence of SNHL in this group is 25%. In half of the cases, SNHL is progressive, with bilateral or unilateral hearing impairment developing with equal probability [5].

Other intrauterine infections are currently much less frequent causes of congenital hearing disorders (rubella due to successful immunization, toxoplasmosis, syphilis due to timely detection and treatment) [6]. There is no convincing evidence regarding the role of herpes virus infection in the development of congenital hearing loss [7].

The main risk factors for permanent congenital and perinatal hearing loss include [1,8]:

- presence of close relatives with childhood-onset hearing impairments

- presence of syndromes or genetic disorders associated with hearing impairments

- anomalies of the auricle, external auditory canal, other craniofacial anomalies (except for preauricular tags, isolated cleft lip)

- intrauterine infections (cytomegalovirus infection, rubella, toxoplasmosis, syphilis)

- stay in a neonatal intensive care unit for more than 48 hours

- prematurity at 32 weeks of gestation or less, or very low birth weight (less than 1500 g)

- hyperbilirubinemia in the neonatal period with blood bilirubin levels requiring exchange transfusion

- perinatal asphyxia, hypoxia

- severe hypoxic-ischemic/hemorrhagic central nervous system injury (grade II–III cerebral ischemia, grade III–IV intraventricular hemorrhage, periventricular leukomalacia); cerebral palsy, organic CNS lesion; neurodegenerative diseases

- treatment of the child with medications possessing potential ototoxic effects

- extracorporeal membrane oxygenation

Causes of acquired SNHL in childhood include meningitis, viral childhood infections, use of ototoxic drugs (aminoglycosides, diuretics, etc.), head trauma (temporal bone/skull base fracture), and neurodegenerative diseases [9,10].

1.3 Epidemiology of the Disease or Condition

Congenital sensorineural hearing loss (SNHL) is the most common congenital sensory pathology. There is one deaf newborn per 1,000 healthy newborns, and another 2–3 children lose their hearing during the first year of life. Among the newborn population requiring intensive care, 20–40 children per 1,000 have hearing impairments [1,11,12].

With age, the prevalence of permanent hearing impairment in children increases to 3–4 per 1,000 [12,13]. Sensorineural hearing loss accounts for approximately 80–85% of congenital hearing impairment cases. Bilateral hearing impairment accounts for 85–88% of congenital neural hearing loss cases. Acquired SNHL accounts for only 11–12% [2,10,12-14, 117].

According to the Russian Ministry of Health, based on statistical reporting on healthcare utilization, the prevalence of bilateral sensorineural hearing loss among children aged 0–14 years in 2017 was 140 per 100,000 children [15].

1.4 Features of Coding the Disease or Condition

H90.3 – Sensorineural hearing loss, bilateral;

H90.4 – Sensorineural hearing loss, unilateral with normal hearing on the opposite ear;

H90.5 – Sensorineural hearing loss, unspecified.

1.5 Classification of the disease or condition

1. By severity (averaged air-conduction hearing threshold at frequencies of 500, 1000, 2000, and 4000 Hz):

• Grade I – 26–40 dB;

• Grade II – 41–55 dB;

• Grade III – 56–70 dB;

• Grade IV – 71–90 dB;

• deafness – 91 dB or more.

2. By time of onset:

• congenital

  - hearing loss detected from birth

  - detection of a pathological genotype regardless of when hearing loss is detected;

  - acquired

  - resulting from an established cause with subsequent deterioration in sound response and possible regression of age-appropriate speech skills.

  - arising from an unknown cause in the presence of evidence of previously normal hearing

3. By course:

• stable;

• progressive;

• fluctuating;

• with improvement.

4. By acuteness of the disease:

• sudden (idiopathic) – develops within up to 12 hours

• acute – develops within 1–3 days and persists for up to 1 month from the onset of the disease

• subacute – 1–3 months from the onset of the disease;

• chronic (permanent) – more than 3 months or lack of anamnestic data on the onset of the disease.

5. By stage of speech development:

• pre-lingual (dilingual – before speech acquisition);

• post-lingual (postlingual – after speech acquisition);

1.6 Clinical presentation of the disease or condition

The main signs of sensorineural hearing loss are the absence or questionable reaction of the child to ambient sounds and a delay in speech development milestones (emergence of cooing, babbling, first words, simple phrases). Sensorineural hearing loss that develops in the post-lingual period is characterized by speech regression (absence of new words, deterioration of sound pronunciation up to the complete disappearance of oral speech). Older children may themselves report hearing loss, impaired speech intelligibility, and tinnitus.

Among anamnestic data, special attention should be paid to the hearing status of close relatives, the course of pregnancy, gestational age at birth, characteristics of labor, birth weight, Apgar score, the course of the perinatal period, the presence of infectious and somatic diseases, and follow-up care by other specialists.

Physical examination includes otoscopy and examination of other ENT organs to rule out pathological conditions of the ear, nose, and pharynx that disrupt airborne sound conduction. During otoscopy, the external auditory canal is clear, the tympanic membrane is pearly grey, dull, and landmarks are clearly contoured.

2. Diagnostics, medical indications and contraindications for diagnostic methods

Otoscopy must be performed before starting hearing tests. The diagnosis of sensorineural hearing loss in young children is based on the results of objective audiologic testing methods – recording various classes of otoacoustic emissions (OAE), auditory brainstem response (ABR), ASSR testing, acoustic impedancemetry, as well as the evaluation of the child's behavioral reactions to non-verbal and verbal acoustic stimuli. In older preschool and school-age children, auditory function can additionally be assessed using pure-tone audiometry, suprathreshold tests, and speech audiometry.

Criteria for establishing sensorineural hearing loss in children:

1. detection during universal newborn hearing screening of infants with suspected hearing loss, anamnestic data indicating the absence or alteration of the child's behavioral reactions to sounds, delayed or impaired speech development, complaints of hearing loss, and the presence of risk factors for hearing loss and deafness;

2. normal otoscopic findings;

3. elevated ABR thresholds and/or altered ABR peak morphology (recording of microphonic potentials in auditory neuropathy), elevated hearing thresholds according to ASSR testing, absence of OAE recording (OAE recording is possible in auditory neuropathy); elevated hearing thresholds according to pure-tone audiometry or other auditory function assessment tests indicating a sensorineural hearing loss pattern.

Universal Newborn and Infant Hearing Screening Program

Comments: the recommended gold standard for full-term infants is:

- hearing screening for all newborns under 1 month of age (screening stage 1);

- confirmatory audiological diagnosis for children identified by hearing screening, under 3 months of age (screening stage 2);

- initiation of an individual (re)habilitation program for children with confirmed hearing loss under 6 months of age.

In Russia, the universal newborn and infant hearing screening program has been implemented at the state level since 2008 [17-20]. The program protocol prescribes performing OAE recording for all newborns on the 3rd–4th day of life in the maternity hospital as stage 1. If the child is transferred to a neonatal pathology department, stage 1 screening is performed upon discharge from this department. In the absence of a clear response in one or both ears, the child undergoes repeat OAE recording at the outpatient clinic at 1 month of age. Children for whom there are no records of hearing screening, those who did not undergo screening in the maternity hospital, and those born outside an obstetric facility also undergo stage 1 screening at the outpatient clinic.

Based on the results of outpatient testing, children with unregistered OAE in one or both ears are referred to stage 2 at specialized audiology centers/clinics for extended audiological evaluation under 3 months of age. In addition, children with the following risk factors for hearing loss must be evaluated at stage 2 regardless of the results of stage 1:

1. Presence of close relatives with childhood-onset hearing impairment;

2. Syndromes associated with hearing impairment;

3. Craniofacial anomalies (excluding preauricular tags and isolated cleft palate/lip);

4. Intrauterine infections (CMV, rubella, toxoplasmosis, syphilis);

5. Stay in a neonatal intensive care unit (NICU) for more than 48 hours;

6. Prematurity at 32 weeks or less, or very low birth weight (less than 1500 g);

7. Severe hyperbilirubinemia (requiring exchange transfusion);

8. Severe hypoxic-ischemic/hemorrhagic central nervous system (CNS) injury;

9. Administration of ototoxic drugs.

Parents or legal guardians must receive information on key stages of hearing, speech, and language development, as well as information on risk factors for progressive or delayed-onset hearing loss. If a delay in auditory reactions or speech development occurs in a child at any stage, they should be referred for age-appropriate hearing testing.

2.1 Complaints and Anamnesis

Complaints and medical history are described in the "Clinical Presentation" section.

2.2 Physical Examination

Physical examination findings are described in the section "Clinical Presentation".

2.3 Laboratory Diagnostics

Comments: in children with two recessive mutations in the GJB2 gene in the absence of inflammatory changes in the middle ear, hearing recovery should not be expected; hearing thresholds are generally stable. Early initiation of (re)habilitation ensures good outcomes provided all conditions are met.

Comments: the subclinical form of congenital CMV infection accounts for 20% of cases of hearing impairment in newborns. Due to the high incidence of the asymptomatic form of the disease and the likelihood of delayed-onset hearing loss, verification of intrauterine infection is necessary.

2.4 Instrumental Diagnostics

Comments: it is recommended to perform hearing screening at the outpatient clinic for children who lack records of screening, those who did not undergo screening in the maternity hospital, and children born outside of an obstetric facility, under the age of 3 months. When seeking medical care at a later age due to a doubtful response of the child to sounds, referral for a comprehensive audiologic evaluation at specialized centers is recommended.

Comments: delayed-onset or progressive hearing loss is likely to develop in the high-risk newborn population.

Comments: ABR recording is one of the main methods for assessing auditory function in children. The study is performed during natural sleep. Performing the study under pharmacological sedation is acceptable in facilities equipped with an intensive care unit.

Determination of ABR thresholds in each ear is performed via click stimulation and/or wideband Chirp stimuli. Thresholds should be determined in steps of no more than 10 dB. It is advisable to analyze ABR morphology, as well as to evaluate interpeak intervals, absolute and relative latency periods in response to suprathreshold stimulation levels.

At suprathreshold stimulation levels of air-conducted wideband stimuli (clicks), it is advisable to use opposite stimulation polarities (condensation and rarefaction) and record waveforms into different memory segments in order to optimize the recording of the cochlear microphonic (CM). This allows diagnosing auditory neuropathy or other forms of auditory pathway pathology. To avoid stimulus artifact contamination of the cochlear microphonic, the use of insert earphones is recommended. To verify the CM, a test with an occluded (kinked) sound tube is recommended.

Obtaining maximum frequency-specific information whenever possible (ASSR, tone bursts, or frequency-specific Chirp tones) is important for determining the degree of hearing loss and has practical application in hearing aid fitting. In addition to the click or high-frequency stimulus, it is recommended to record ABRs to tone bursts at 1000 Hz or 500 Hz.

The ASSR recording method cannot be used in isolation, and this method is always preceded by recording ABRs to a click and/or wideband Chirp stimulus. ASSR recording is not used (is uninformative) in children with a confirmed diagnosis of auditory neuropathy. Diagnosis of this pathology is based on data from ABR, OAE, and cochlear microphonic recordings.

Comments: to obtain more detailed frequency-specific information, OAE recording should also be performed at normal ABR thresholds recorded with click stimulation.

Comments: in children under 6 months of age, tympanometry is performed using a high-frequency probe tone (usually 1000 Hz). In premature infants, high-frequency tympanometry is indicated up to 8–12 months of age.

Comments: the child is seated together with a parent in a sound-treated booth such that the loudspeakers (sound columns) are positioned on both sides of the child. Above or below the speakers are mechanical toys that can move or light up (sometimes instead of a toy, a monitor displaying changing pictures is placed to the side of the subject). A frequency-modulated ("warble") tone of 1000 Hz with an intensity of 50–60 dB nHL (if normal hearing is assumed) or an intensity of 40 dB above the assumed threshold (in case of hearing loss) is presented in the free sound field, or via insert/supra-aural earphones or a bone vibrator, and if the child turns toward the sound stimulus, the toys are activated. If the child does not pay attention to the sound, the intensity is increased until a response from the child is obtained. It is necessary to ensure that the child associates the perception of sound with the start of movement (or illumination) of toys or pictures. The test is repeated at frequencies of 250–4000 Hz.

The technique for determining the hearing threshold corresponds to the technique used in traditional audiometry. If earphones can be used during the child's evaluation, thresholds are measured for each ear separately; if not (i.e., testing was performed in the free sound field), the obtained results should be considered as a response from the better-hearing ear.

Comments: thresholds are determined at frequencies of 125–8000 Hz for each ear separately. Testing is performed for air and bone conduction. The child is instructed to put a ring on a stacking tower peg or perform other similar actions with a toy when they hear a sound. Testing begins at 1000 Hz at an intensity 30–40 dB above the assumed hearing threshold. The technique for determining the hearing threshold corresponds to the technique used in traditional audiometry.

Comments: thresholds are determined at frequencies of 125–8000 Hz for each ear separately. Testing is performed for air and bone conduction. If necessary and technically feasible, extended high-frequency audiometry up to 16–20 kHz is performed (including for monitoring ototoxicity, assessing noise exposure on the auditory organ, etc.).

Comments: speech testing provides information on speech reception and recognition thresholds. The detection of tone-speech dissociation may indicate auditory neuropathy spectrum disorder (ANSD) as well as central auditory processing disorders (CAPD). This helps to refine the diagnosis, develop a (re)habilitation program, and evaluate the efficacy of hearing aids and overall (re)habilitation. During open-set speech audiometry, the child repeats the heard word; in closed-set format, the child points to it on a picture. Testing can be performed using headphones or in a free sound field with loudspeakers (sound columns). When testing with headphones, words are presented to the right ear, the left ear, or both ears simultaneously. Testing can be conducted with descending speech intensity to determine speech recognition thresholds or at a fixed speech stimulus intensity. If speech material is delivered via loudspeakers in a free sound field or via headphones to both ears simultaneously, the result should be considered an assessment of the better-hearing ear. To evaluate the efficacy of hearing aids (cochlear implants), the test is performed in a free sound field with one or two hearing devices (hearing aids/implants).

Comments: used to determine the site of lesion in the auditory pathway. Identifying disorders of the central auditory system affects diagnostic and (re)habilitation management.

2.5 Other Procedures

Comments: three-dimensional imaging methods are essential in diagnosing congenital malformations of the auditory system that require a specialized rehabilitation program.

Comments: the assessment of the child's auditory-verbal development is carried out by a deaf-and-speech educator (surdopedagogue) using one or more questionnaires. Questionnaires must correspond to the child's age, developmental level, and testing objectives; the following scales may be used [45–50]:

- LittlEARS Auditory Questionnaire for infants;

- Scale "Levels of Auditory Perception in Hearing-Impaired Children";

- Scale "Levels of Speech Intelligibility in Hearing-Impaired Children";

- PEACH (Parents' Evaluation of Aural/Oral Performance of Children);

- MacArthur Communicative Development Inventories (CDI).

Surdopedagogical testing includes assessing the child's auditory perception of speech sounds using observational methods and standardized tests from the "Evaluation of Auditory and Speech Development of Children with Cochlear Implants/Hearing Aids" (EARS) test battery, as well as evaluating speech, language, and communicative development [45].

The assessment of the child's overall development, including cognitive and communicative domains, is carried out by a specialist (preferably a psychologist). The following questionnaires and scales may be used for this purpose [51–56]:

- KID scale for assessing child development up to 16 months;

- RCDI-2000 scale for assessing child development from 1 year 2 months to 3 years 6 months;

- M-CHAT questionnaire for children aged 16 to 30 months, aimed at assessing communication and interaction patterns;

- Wechsler Intelligence Scale for Children (WISC) to assess the level of intellectual development in children over 4 years old;

- Raven's Progressive Matrices to assess the level of intellectual development in children over 5

years old.

To identify additional developmental disorders in the child and design an individualized auditory (re)habilitation program, the use of one or more questionnaires and tests is recommended. The assessment results can subsequently be used during patient follow-up to evaluate the efficacy of auditory (re)habilitation.

Comments: 24–40% of children with hearing loss have additional disorders that affect the efficacy of their (re)habilitation [17, 57, 58]. The (re)habilitation program for a child with hearing loss must be comprehensive and take into account the child's various needs.

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

3.1 Conservative Treatment

Comments: glucocorticoids have a universal therapeutic effect in sudden sensorineural hearing loss (SSNHL) regardless of etiology. For example, #methylprednisolone** may be used at a dosage of 1 mg/kg per day (once daily) orally for 14 days with a gradual dose taper every 4 days [60–62].

3.2 Surgical Treatment

Comments: currently, the only effective method of rehabilitation for patients suffering from grade IV sensorineural hearing loss and deafness is cochlear implantation. This represents a set of audiologic, surdopedagogical, psychological, and surgical measures aimed at the complete or partial restoration of impaired functions and/or compensation for lost functions of the peripheral auditory system (the cochlea), maintaining or developing auditory-verbal function, preventing and reducing potential disability, improving quality of life, preserving the patient's work capacity, and facilitating social integration.

Priority in providing this type of care is given to young children (under 3 years of age). Cochlear implantation in individuals over 3 years of age with a lack of auditory-verbal skill development is possible based on the decision of the medical commission of the center performing the surgical treatment, with mandatory consultation and evaluation of the prospects for hearing and speech skill development by an otolaryngologist-audiologist and following medical and educational observation.

The question of indications for CI is decided after consistent binaural use of hearing aids for 3–6 months with no benefit from hearing aids, according to the conclusion of the surdopedagogical commission of the center performing the surgical treatment, and provided there is family motivation. Children with bilateral grade IV sensorineural hearing loss or deafness due to confirmed hereditary hearing loss (mutations in the GJB2 gene and other genes), as well as those with bilateral grade IV sensorineural hearing loss or deafness resulting from meningitis (meningoencephalitis), may be referred for CI bypassing the hearing aid trial stage.

Indications for cochlear implantation:

- Chronic bilateral grade IV sensorineural hearing loss or deafness with average hearing thresholds of 80 dB or worse in the frequency range of 500–4000 Hz according to comprehensive hearing assessment data, including auditory brainstem response (ABR) and ASSR testing (taking into account correction factors).

- Hearing thresholds in a free sound field with optimally fitted hearing aids (binaural amplification) exceed 40 dB at frequencies of 0.5–4 kHz.

- Lack of significant improvement in speech hearing perception from the use of optimally fitted hearing aids in severe to profound bilateral sensorineural hearing loss, after at least 3–6 months of hearing aid use, according to the conclusion of the audiologic-pedagogical commission. This period may be shortened in children with a confirmed homozygous or compound heterozygous mutation 35delG in the GJB2 gene.

- Chronic bilateral sensorineural hearing loss of the auditory neuropathy type of any degree when hearing aid fitting is ineffective with continuous use of hearing aids for at least 3–6 months, according to the conclusion of the audiologic-pedagogical commission of the center performing the surgical treatment.

Absolute contraindications for cochlear implantation:

- Complete aplasia of the inner ear (Michel anomaly).

- Complete obliteration of the cochlea.

- Presence of severe somatic, neurological, and mental disorders precluding surgery under general anesthesia, cochlear implant (CI) use, and postoperative auditory-verbal rehabilitation.

Relative contraindications for cochlear implantation (determined by the medical board of the Center performing the surgical treatment):

- Lack of motivation or socio-psychological capacity for a long-term rehabilitation process on the part of the patient (parents, legal representative, or authorized proxy).

- Partial cochlear obliteration (as determined by the medical board of the center performing the surgical treatment).

- Anomalies of cochlear and auditory nerve development (as determined by the medical board of the center performing the surgical treatment).

- Acute or chronic inflammatory diseases of the ENT organs in the acute stage.

- Retrocochlear pathology (as determined by the medical board of the center performing the surgical treatment).

Bilateral cochlear implantation is a set of audiological, surdopedagogical, psychological, and surgical procedures for installing two cochlear implant systems in both ears. Bilateral cochlear implantation is performed simultaneously (during a single surgical procedure) or sequentially (during two surgical procedures).

Bilateral cochlear implantation provides:

- bilateral stimulation of the auditory pathways and auditory centers; the ability to localize the sound source in space and perceive sounds from different directions;

- improved speech intelligibility in noise and when communicating with multiple speakers;

- better dynamics of auditory and speech development, since speech sounds louder and

clear, no "head shadow" effect, the process of sound perception requires less effort;

- preservation of sound perception function in the event of failure of one implant.

Indications for bilateral cochlear implantation:

- Indications for simultaneous bilateral cochlear implantation:

- Chronic bilateral sensorineural hearing loss grade IV and deafness following meningitis (with a medical record extract provided) and by decision of the medical commission of the center performing the surgical treatment.

- I. Indications for sequential bilateral cochlear implantation:

- Chronic bilateral grade IV sensorineural hearing loss and deafness with radiological signs of cochlear ossification/obliteration due to temporal bone fractures and otosclerosis, as determined by the medical board of a center performing surgical treatment.

- Combined or isolated inner ear developmental anomalies, as determined by the medical board of a center performing surgical treatment.

- In the presence of the specified inner ear developmental anomalies, the number of spiral ganglion cells is reduced, and unilateral cochlear implantation may be insufficient to achieve a satisfactory effect of auditory-verbal rehabilitation.

- Presence of visual impairments in the patient (low vision and blindness) to provide better spatial orientation and speech perception in noise, since these patients are unable to perceive speech audio-visually.

Presence of positive dynamics in hearing and speech development, successful social adaptation, and active psycho-educational rehabilitation after unilateral cochlear implantation, given the high motivation of patients and their parents (legal representatives, proxies), based on the conclusion of the medical board of the center performing the surgical treatment. At the same time, the volume of sequential binaural cochlear implantation should not exceed 20% of the annual volume of this type of medical care provided by the institution.

The warranty period for the internal part of the cochlear implantation system (implant) must be at least 10 years, and for the external part (speech processor) at least 5 years.

Revision cochlear implantation (reimplantation)

Indications for reimplantation:

Repeat surgical treatment (reimplantation) is performed based on the decision of the specialized board of the operating clinic.

In the event of failure of the internal part of the cochlear implantation system (implant) during the warranty period, the manufacturer, upon the application of the medical institution where the patient applied for diagnostics, conducts a preliminary examination and issues an initial conclusion with recommendations, including the need for removal of the internal part of the cochlear implantation system (explantation), and also provides the medical institution with the internal part of the cochlear implantation system for repeat surgical treatment.

The final fact of malfunction of the internal part of the cochlear implantation system is established after a technical examination of the extracted implant, which is carried out by the manufacturer or an independent expert at the applicant's expense. The manufacturer sends a written final conclusion to the medical institution regarding the condition of the internal part of the cochlear implantation system, containing information on the possible causes that led to the failure of the cochlear implant.

Comments: indications – bilateral deafness caused by auditory nerve damage (patients with type 2 neurofibromatosis, after traumatic brain injury); significant or complete cochlear ossification; auditory nerve or cochlear aplasia. Contraindications: presence of comorbid conditions preventing surgical treatment; lack of motivation for surgery and postoperative auditory-verbal (re)habilitation, or lack of socio-psychological resources for a long-term (re)habilitation process in the patient, the patient's parents, family members, or guardians.

4. Medical Rehabilitation and Sanatorium-Resort Treatment

An individual rehabilitation program must be established for a child with confirmed hearing loss by 6 months of age (corrected age in premature infants). Rehabilitation measures may include: various types of hearing aids (correction of auditory function); deaf-and-dumb education, speech therapy, and psychological assistance to the child; informational and psychological support for the family and the child; social support for the child; follow-up care (dispensary observation). When applying for disability status, a medical and social expert evaluation report is prepared for inclusion in the individual rehabilitation and habilitation program (IPRA), indicating rehabilitation measures and assistive rehabilitation devices (ARD). The individual assistance program should begin immediately after the hearing impairment is detected in the child.

Comments: hearing aid fitting is recommended for children with bilateral chronic sensorineural hearing loss with average air-conduction hearing thresholds (at 500, 1000, 2000, and 4000 Hz) of 31 dB or worse in the better-hearing ear. In cases of bilateral grade I chronic hearing loss (average hearing threshold 26–40 dB) in the better-hearing ear (or both ears) and correctable unilateral hearing loss, additional examination is indicated (speech testing and/or psychoeducational assessment), based on the results of which a final decision is made regarding the feasibility of hearing aid fitting in each specific case. In cases of uncorrectable unilateral hearing loss (deafness), the use of remote microphone systems may be recommended.

Contraindications to hearing aid fitting:

- Acute hearing loss, which may indicate an acute condition or an exacerbation of a chronic condition requiring thorough diagnosis and alternative treatments.

- For air-conduction hearing aid fitting: purulent-inflammatory diseases of the external or middle ear in the acute stage, which may be aggravated by occlusion of the external auditory canal with an earmold.

- In a number of cases, conditions accompanied by severe dizziness may be a contraindication to hearing aid fitting.

Criteria for determining indications for hearing aid fitting should be based on the following examinations:

- Electrophysiological assessment of auditory sensitivity (click- and frequency-specific stimulus ABR), auditory steady-state response (ASSR);

- Determination of behavioral thresholds using standard audiometric techniques appropriate for the child's age (visual reinforcement audiometry, play audiometry, standard pure-tone audiometry).

Comments: validation must involve multiple methods – one or two audiological methods, one questionnaire, and deaf education (surdopedagogical) testing.

- Audiological methods:

⮚ assessment of pure-tone hearing thresholds with hearing aids or auditory implants in a free sound field for each ear;

⮚ assessment of speech perception using speech audiometry with hearing aids or auditory implants in a free sound field for children over 5 years of age with an adequate level of speech development (testing format – open or closed-set word choice – depends on the child's level of speech development); speech tests are used both in quiet and against background noise.

- Questionnaires for parents/guardians/child/teachers [45,46,70]:

⮚ the deaf educator (surdopedagogue) assesses the auditory and speech development of the child using hearing assistive technology, employing observation, testing, and parent questionnaires. For this purpose, the questionnaires provided in Section 2.2 are used, as well as the LittlEARS / Auditory Integration Scale questionnaire.

⮚ to assess the level of speech and hearing skills development in a child using hearing assistive technology, standardized tests from the "Evaluation of Auditory and Speech Development of a Child with CI/HA" (EARS) test battery are used.

The evaluation results are recorded in the patient's medical documentation. Criteria for effective hearing aid fitting: positive dynamics in the child's auditory and

speech/communicative development, an increase in the percentage of word recognition in quiet (if testing is feasible).

In case of unsatisfactory validation results, an analysis of potential causes for low (re)habilitation efficacy is performed (analyzing the diagnostic stage; treatment strategy selection; choice of assistive device for hearing correction; surgical stage, if performed; device fitting; psychoeducational work, etc.). These causes are minimized or eliminated whenever possible. Afterward, re-validation is performed in 1-3 months. The main implemented changes and the results of re-validation are documented in the patient's medical records.

Assistive devices for hard-of-hearing/deaf patients

When selecting a hearing aid, the possibility of using remote microphone systems should be considered. All children using hearing aids, cochlear implants, or middle ear implants (or considered candidates for hearing aid fitting) are potential candidates for assistive listening technologies. These technologies are particularly relevant for school-age children, as well as children with severe hearing loss, auditory neuropathy spectrum disorder (ANSD), and central auditory processing disorders. The choice of an assistive listening device is made individually depending on the child's age, degree of hearing loss, type of hearing correction, and type of hearing aid or speech processor. When pairing the device with a hearing aid or speech processor, it is necessary to configure the initial program to allow prompt or automatic reception of the signal from the transmitter upon activation. One of the key factors in the successful use of assistive listening devices is teachers' awareness of operating rules and their motivation. Electroacoustic verification of the individual system is required to assess its performance quality.

List of assistive devices for hard-of-hearing/deaf patients:

- personal and group FM systems

- induction loop systems

- digital systems (remote microphones, wireless streamers)

- telephones with a built-in signal amplifier

- televisions for receiving closed-captioned programs (with teletext) – for children with automated reading skills

- various vibrational, visual, and vibro-visual sound signaling devices

- text telephones – for children with automated reading skills

Initial stage of hearing aid fitting and adaptation to hearing assistive technology

Parents must be provided with comprehensive oral and written information on the care and use of hearing assistive technology [46,48,70].

During the adaptation stage, the participation of a deaf educator is necessary to assess the child's auditory reactions to sounds with and without assistive devices, to develop the child's auditory perception and speech, to identify problems with device usage, and to teach parents how to develop the child's hearing and speech at home. Close interaction between specialists and parents is essential, keeping them informed about the upcoming stages of the care program and involving the family in the hearing-impaired child's (re)habilitation process. The further (re)habilitation program is developed and implemented by a multidisciplinary team of specialists with the active participation of the family.

Timeframes for the hearing aid/cochlear implant fitting and adaptation stage:

When drawing up the patient's (re)habilitation program, an individual schedule of follow-up visits with specialists is determined.

Following the initial fitting, during the hearing aid adaptation period (usually within 1-3 months), at least 1-4 follow-up consultations with an otorhinolaryngologist-audiologist and a deaf educator are conducted. These include assessing the effectiveness of the fitting (validation), identifying problems with hearing aid use, and, if necessary, adjusting the hearing aid settings based on validation results as well as observations of the child's reactions by parents and specialists. The frequency of follow-up adjustments is determined individually in each case, depending on the child's age, diagnosis, device type, and other factors. During the first two years of hearing aid use, setting checks are recommended at least once every 3 months. Once optimal fitting is achieved, follow-up monitoring by an otorhinolaryngologist-audiologist is carried out. The main consultation results are recorded in the patient's medical documentation. The frequency of follow-up is individual and depends on the child's age, level of auditory and speech skills development, and comorbid conditions. It is recommended every 3 months for children under 2 years of age, every 6 months up to 6–7 years of age, and subsequently 1–2 times a year. Children using hearing aids/cochlear implants are indicated for follow-up clinical monitoring (dispensary observation) by an otorhinolaryngologist and an otorhinolaryngologist-audiologist.

Assessment of functioning in children with hearing impairment. Rehabilitation status. Rehabilitation diagnosis.

Assessment of functioning in children with hearing impairment is carried out from the perspective of the International Classification of Functioning, Disability and Health (ICF).

WHO has developed an ICF core set for individuals with hearing loss. The form of the document was approved by the WHO Committee on August 23, 2019, No. 88.

The ICF core set is the maximally optimal set of significant ICF domains used in developing a rehabilitation program for patients with hearing loss. The ICF core set contains a concise list of ICF categories deemed relevant for describing people with a given health condition (e.g., sensorineural hearing loss) or within a health-related context.

The severity of identified persistent hearing impairments and other functions is assessed as a percentage (Appendix A3, Fig. 1).

Comments: After receiving the results of the child's audiological examination, the medical organization providing primary health care and specialized medical care (most often a district polyclinic) proceeds to issue a referral for the MSE (Appendix A3, Fig. 2 and Fig. 3). For all citizens recognized as disabled, an individual rehabilitation and habilitation program (IRHP) is developed for the disabled person (disabled child). Federal state MSE institutions send an extract from the IRHP to the relevant executive authorities, local self-government bodies, and organizations, regardless of their legal forms and ownership types, entrusted with carrying out rehabilitation or habilitation measures provided for by the IRHP (Appendix D, Fig. 4). The executive body of state power of a constituent entity of the Russian Federation in the relevant field of activity, the regional branch of the Social Fund of Russia, and in certain cases ‒ the administration of inpatient-type institutions or institutions of the Federal Penitentiary Service organize work on the implementation of IRHP measures.

Psycho-educational support for children with hearing impairment

Comments: psycho-educational support for a child with hearing impairment includes:

- assessment of the level of hearing and speech development at the stage of hearing loss diagnosis;

- adaptation of the child to wearing hearing aids/cochlear implants/bone conduction implants and perceiving sounds/speech through hearing assistive devices;

- assistance of the surdopedagogue to the audiologist-prosthetist in determining the optimal fitting parameters of hearing aids/cochlear implants/bone conduction implants during initial fitting, adjustment correction, and replacement of hearing aids/cochlear implants/bone conduction implants, creating conditions for the constant use of hearing assistive devices by the child;

- development/restoration of auditory perception of sounds and speech using hearing aids/cochlear implants/bone conduction implants in a child after hearing aid fitting during corrective developmental sessions with a surdopedagogue/speech-language pathologist/medical speech-language pathologist;

- development/correction of oral speech, language system, communication skills, and other higher mental functions in a child with hearing aids/cochlear implants/bone conduction implants, taking into account additional primary developmental disorders identified in the child during corrective developmental sessions with a surdopedagogue/speech-language pathologist;

- surdopedagogical assessment of the effectiveness of hearing aid fitting and the child's use of hearing assistive devices for the development of auditory perception and oral speech, including in daily situations, identifying problems with the use of hearing aids/cochlear implants/bone conduction implants, and assistance in solving these problems;

- prevention and correction of secondary disorders in the development of mental functions in a child with hearing loss;

- formation of compensatory behavioral strategies in a child with hearing impairment during speech perception in complex acoustic conditions;

- psychological assistance to a child with hearing impairment studying in various educational settings (specialized correctional and mainstream schools);

- psychological support and assistance to the child's family members in their ability to make informed decisions regarding (re)habilitation, development, education, and upbringing of a child with hearing impairment;

- counseling the child's family members on care and the use of hearing assistive devices, increasing the effectiveness of hearing aids for developing auditory-speech perception, oral speech, communication and other skills in the child, communication issues with the child, including, if necessary, alternative communication means, child development and education during daily routines, and creating favorable conditions for the development of auditory, speech, language, and communication skills in a hearing-impaired child;

- training the child's family members in independent sessions for the development of

- auditory-speech perception, oral speech, language, and communication skills in a child with hearing aids/cochlear implants/bone conduction implants at home.

For children with prelingual hearing loss of various types and degrees using all types of hearing aids (including during hearing aid replacement), as well as cochlear implants/bone conduction implants, it is advisable to provide:

- Corrective developmental sessions with a surdopedagogue/defectologist (development of auditory and audiovisual speech perception in a child with hearing aids/cochlear implants/bone conduction implants; determination of optimal fitting parameters and usage modes of hearing assistive devices; development and correction of the articulatory aspect of oral speech, language system, and speech communication skills; assessment of the effectiveness of hearing aid fitting using surdopedagogical methods; training the child and parents/guardians in the use and care of hearing assistive devices; counseling parents/guardians of the child on issues related to increasing the effectiveness of hearing aids; if necessary, training the child and family members in the use of alternative communication means) – 1–5 times a week for 1–7 years, depending on the degree of hearing loss and the type of hearing assistive device;

- Corrective developmental sessions with a medical speech-language pathologist/defectologist (speech-language pathologist): diagnostic procedures; individual and group corrective sessions aimed at:

• formation, development, and realization of their speech abilities and skills, taking into account the capabilities and individual development characteristics of each child;

• implementing activities to stop (minimize) undesirable, socially unacceptable behavior of patients;

• social and speech adaptation of the patient in a new or unfamiliar communicative environment;

• reducing the risk of progression of speech disorders together with the patients' parents (or their legal representatives);

• interactive inclusion of parents (legal representatives) of patients in developmental activities.

• Individual speech therapy sessions:

• diagnostic and consultative (with patients' parents and legal representatives) activities on issues within the competence of the speech-language pathologist;

• performing speech therapy massage (category: preschool and early school-age patients;

• conducting speech therapy exercises/drills;

• conducting preparatory work when activating patients' speech capabilities for participation in various socio-communicative developmental activities.

• Group speech therapy sessions:

• conducting correctional and developmental sessions, including those with elements of phonetic / speech therapy rhythmics;

• preparing developmental activities (determining content, type, selection of speech material taking into account the individual capabilities of each patient) of a developmental and socio-communicative orientation (thematic, festive, cognitive-entertainment, etc.).

- Consultations and sessions with a psychologist/educational psychologist on the development and correction of higher mental functions in the child (thinking, memory, attention, emotional-volitional processes, etc.), development of play activity corresponding to the stage of the child's age development; correction of disorders in the emotional-volitional and motivational spheres of the personality; formation of compensatory behavioral strategies, pre-speech and speech communicative skills in the child; counseling parents/guardians of the child on issues related to the development, education, upbringing, and psychological support of a child with hearing impairment; - 1-50

- consultations/sessions (as recommended by a pediatrician, otorhinolaryngologist/audiologist, and deaf-and-dumb educator [deaf-pedagogue]);

- When organizing auditory-verbal rehabilitation courses for children with hearing aids (HA/HCP) on the basis of an audiological center, the structure of the courses should include musical stimulation sessions, as well as group sessions with children on the development of hearing, speech, and speech communicative skills between individual sessions with the deaf-pedagogue, speech therapist, and psychologist.

Children with postlingual sensorineural hearing loss of various types and degrees using all types of hearing aids (including when replacing hearing aids), as well as cochlear implants / auditory brainstem implants, should preferably be provided with:

- Consultations and sessions with a deaf-pedagogue / teacher-defectologist (deaf-pedagogue) (adaptation of the child to the perception of sounds and speech using hearing aids/cochlear implants/auditory brainstem implants, development of auditory and audio-visual speech perception using hearing aids/HCP, determination of optimal fitting parameters and usage modes of hearing aids/HCP, assessment of the effectiveness of hearing-aid fitting using deaf-pedagogical methods, training the child and parents/guardians in the care and use of hearing aids/HCP) – 1-100 sessions depending on the degree of hearing loss and the type of hearing aid/HCP;

- Correctional and developmental sessions with a medical speech therapist / teacher-defectologist (speech therapist): diagnostic procedures; individual and group correctional sessions aimed at restoring speech skills, correcting speech defects, and preventing potential secondary speech disorders, using such procedures as speech therapy massage, articulation gymnastics, speech rhythmics (eurythmy), kinesiotaping, myofunctional therapy, phonetic rhythmics, eliciting/induction/automation of

- sound pronunciation, formation of phrasal and connected speech, normalization of prosodic characteristics of speech, consultative measures for parents/guardians to involve them in the correction process - 1-100 sessions depending on the degree of hearing loss and the type of hearing aid/HCP;

- Consultations with a psychologist / educational psychologist (correction of the psychological consequences of hearing loss in the child; formation of compensatory behavioral strategies in the child; development of play activity appropriate to the stage of the child's age development; correction of disorders in the emotional-volitional and motivational sphere associated with hearing loss; counseling the child's parents/guardians on issues related to the development, education, upbringing, psychological support of a child with hearing loss, and effective communication with the child in daily situations) – 1-10 consultations (as recommended by the audiologist and deaf-pedagogue).

Physical rehabilitation methods, sanatorium-resort treatment, medical indications and contraindications for the use of physiotherapy, massage, exercise therapy (physical therapy), and natural healing factors

Comments: local galvanization (galvanization for diseases of the peripheral nervous system) and drug electrophoresis (intra-aural electrophoresis of medicinal products for diseases of the organ of hearing), electrical stimulation, amplipulse therapy (exposure to an alternating electrostatic field), magnetotherapy (exposure to magnetic fields), phototherapy (light therapy with short-wave ultraviolet radiation of the external ear), laser therapy (low-intensity laser therapy [intravenous blood irradiation]), ultrasonic therapy (endoauricular exposure to low-intensity laser radiation for diseases of the hearing organs), vibrotherapy/hydrovibrotherapy (vibrational exposure), oxygen therapy. A course of 8-10 procedures, daily or every other day.

Physiotherapy, massage procedures, and exercise therapy sessions are prescribed by a physical therapy physician, exercise therapy physician, or medical rehabilitation physician.

General contraindications to the prescription of physical factors:

✔ general severe condition of the child;

✔ hyperthermia;

✔ pronounced intoxication;

✔ grade 2-3 hypotrophy;

✔ blood diseases, increased bleeding tendency;

✔ active-phase tuberculosis and during the period of tuberculin test conversion;

✔ grade 2-3 circulatory failure;

✔ pronounced renal and hepatic insufficiency;

✔ malignant neoplasms.

✔ Specific contraindications to the prescription of physical factors:

✔ individual intolerance or hypersensitivity to

the physical factor;

✔ diseases and pathological conditions in which physiotherapeutic

intervention causes undesirable and adverse effects.

✔ in children using a cochlear implant system, brainstem

implant, or middle ear implant, the following physiotherapy procedures are contraindicated: magnetotherapy, electrotherapy to the head and collar area to avoid damage to surrounding tissues and the internal part of the implant; hydrokinesiotherapy procedures are performed with the external part of the implant removed.

Comments: perform balance and equilibrium training exercises (balance therapy), and breathing exercises. Therapeutic exercise sessions are conducted in courses for 3 months, 3 times a week for 30–40 minutes. Additionally, terrain cure (terrenkur) for at least 20 minutes daily is recommended.

Comments: therapeutic massage (classical, acupressure, vacuum) of the parotid, occipital, and collar regions is performed daily, with a course of 8–10 procedures.

Comment: sanatorium-resort treatment is carried out once a year according to indications.

Sanatorium-resort treatment of the pediatric population is carried out in accordance with Order No. 664n dated October 13, 2022. VI.I Medical indications for sanatorium-resort treatment of the pediatric population with diseases of the ear and mastoid process (Class VIII according to ICD-10).

The purpose of sanatorium-and-spa treatment for individuals with hearing impairments, in accordance with Federal Law No. 323-FZ dated November 21, 2011, "On the Fundamentals of Protecting the Health of Citizens in the Russian Federation," is the full or partial restoration of impaired functions and/or compensation for lost functions of the affected organ or body system, the maintenance of bodily functions during the resolution of an acutely developing pathological process or an exacerbation of a chronic pathological process, as well as the prevention, early diagnosis, and correction of potential dysfunctions of damaged organs or body systems, the prevention and reduction of the degree of potential disability, the improvement of quality of life, the preservation of the patient's working capacity, and their social integration into society.

5. Prevention and Dispensary Observation

Comments: throughout the rehabilitation process, parents, family members, and other individuals assisting the child with hearing aids and cochlear implants must receive ongoing professional support.

Recommended frequency of follow-up audiological evaluations:

- during the first two years of life and/or the first two years of hearing aid use – at least once every three months;

- age 2–5 years and/or the second to third year of hearing aid use – every 3–6 months;

- older than 5 years and/or after 3–4 years of device use – 1–2 times a year;

- after cochlear implantation (CI) – in accordance with the clinical guidelines "Rehabilitation of Patients After Cochlear Implantation".

For patients with unilateral hearing loss/deafness, as well as those with hearing loss not requiring amplification, dynamic monitoring of auditory function and language and speech development must be performed every 6 months until the child reaches 3 years of age, and thereafter 1–2 times a year.

For patients in special groups (with developmental anomalies of the cochlea, auditory nerve, or other temporal bone structures, cochlear ossification, auditory neuropathy, comorbid conditions, prolonged duration of deafness, hearing loss acquired after traumatic brain injury, and other complications) who may encounter difficulties at various stages of hearing aid fitting and cochlear implantation, an individualized schedule for condition testing and hearing aid/CI processor programming may be used [47,101].

Comments: in children with congenital CMVI, sensorineural hearing loss may develop with a delayed onset. To ensure timely detection of sensorineural hearing loss, all children with congenital CMVI require periodic hearing monitoring.

Recommended frequency of audiological evaluations in congenital CMVI [8,105]:

- every 3 months under 12 months of age;

- every 6 months at 13–36 months of age;

- every 12 months from 3 to 6 years of age.

Follow-up assessments and monitoring during hearing aid fitting must include:

- Behavioral audiometry, including air and bone conduction testing;

- Acoustic impedance audiometry to assess middle ear function;

- Adjustment of correction parameters based on the obtained audiometric data and the child's age-related changes;

- Electroacoustic verification or listening check of the hearing aid (if technically feasible);

- Assessment of the condition of the custom earmold;

- Real-ear probe microphone measurements, real-ear-to-coupler difference (RECD) measurements, and adjustments based on these measurements (custom earmolds are replaced as the child grows) (if technically feasible);

- Measurements of functional gain.

Replacement of a Hearing Aid

The established period of use for hearing aids, in accordance with Order No. 107n of the Ministry of Labor and Social Protection of the Russian Federation dated March 5, 2021, "On Approval of Periods of Use for Rehabilitation Equipment, Prostheses, and Orthotic Products," is at least 4 years; the period of use for a custom earmold for a hearing aid is at least 1 year; for a teletext television for receiving closed-captioned programs – at least 7 years; for telephone devices with video communication, navigation, and text output functions – at least 7 years; and for light and vibration sound indicators – at least 5 years.

Repair of rehabilitation equipment is provided free of charge during the warranty period in accordance with Order No. 605n of the Ministry of Labor and Social Protection of the Russian Federation dated August 30, 2019.

Replacement of rehabilitation equipment is carried out upon application in the event of:

- expiration of the established period of use;

- impossibility of repair or need for premature replacement, which must be confirmed by a medical and technical expert report;

- for medical indications in case of deterioration of hearing thresholds not compensated by the existing hearing aid.

Replacement of the speech processor

Replacement of the speech processor is performed 5 years after the date of the previous installation of the cochlear implant speech processor to the patient at the expense of the compulsory medical insurance fund and is carried out in accordance with the requirements of Order of the Russian Ministry of Health No. 178n of April 9, 2015 "On approval of the Procedure for the provision of medical care to the population in the profile 'surdology-otorhinolaryngology'". Order of the Ministry of Health of the Russian Federation No. 526n of September 17, 2014

When replacing the speech processor and during the initial activation of the speech processor, the patient must provide documents regarding the surgical treatment (cochlear implantation), and in the case of a previously performed speech processor replacement, documents confirming this fact. The patient or their legal representative bears personal responsibility for complying with the speech processor replacement schedule.

Follow-up examinations and monitoring after cochlear implantation, taking into account the child's age characteristics, should include:

- Deaf education testing of the patient;

- Pure-tone threshold audiometry (play audiometry) in a free sound field;

- Recording of the auditory nerve action potential using neural response telemetry;

- Speech audiometry in a free sound field;

- Speech processor programming;

- Follow-up consultation with a deaf-and-dumb educator.

6. Organization of Medical Care

Indications for hospital admission to a medical organization:

- sudden sensorineural hearing loss;

- replacement of the CI speech processor (in a day hospital setting);

- performing an objective audiological examination under general anesthesia (in a day hospital setting);

Indications for patient discharge from a medical organization

- recovery;

- improvement.

7. Additional Information

Hearing loss has major social, medical, and psychological significance. Uncompensated hearing loss leads to a reduced quality of life and lower educational attainment, and limits career choices in proportion to the degree of hearing loss, depending on the child's age and level of social activity.

While acute sensorineural hearing loss carries a probability of hearing restoration through therapeutic intervention, in chronic sensorineural hearing loss, from an evidence-based medicine perspective, the possibility of improving hearing thresholds with drug therapy is extremely low.

Regarding chronic sensorineural hearing loss, it is more accurate to speak of treating a child with hearing loss for comorbid somatic pathology they may have. Medical treatment of cardiovascular diseases, nervous system disorders, endocrine pathology, and immune status abnormalities simultaneously serves as both treatment and prevention of the progression of chronic sensorineural hearing loss in these children.

In the presence of vestibular disorders and subjective tinnitus associated with chronic sensorineural hearing loss, pathogenetic and symptomatic treatment is administered.

In a child with sensorineural hearing loss accompanied by visual impairment and other disabling conditions, the indications for hearing aid fitting are broadened, and the performance requirements for assistive technology devices increase.

The inability to achieve effectiveness using hearing aids and cochlear implants necessitates the implementation of an alternative communication method—learning the basics of the fingerspelling alphabet and sign language (sign language interpretation).

Criteria for Evaluating the Quality of Medical Care

References

Appendix A1. Working Group Composition

Adnan S. Yunusov^1,4, D.Sc. (Med.), Professor, Member of the Medical Association, no conflict of interest.

Anton S. Machalov^1,3,4, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Maria Yu. Boboshko⁹, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Georgy A. Tavartkiladze⁹, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Leyla S. Namazova-Baranova^4,15, Academician of the RAS, Professor, D.Sc. (Med.), President of the Union of Pediatricians of Russia; Past President of EPA/UNEPSA, no conflict of interest.

Aleksandr A. Baranov^15,21, Academician of the RAS, Professor, D.Sc. (Med.); Honorary President of the Union of Pediatricians of Russia, no conflict of interest.

Tatyana G. Markova^8,10, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Mikhail V. Nikitin¹⁴, D.Sc. (Med.), Chief freelance specialist in sanatorium-resort treatment of the Russian Ministry of Health, no conflict of interest.

Olga V. Karneeva^1,4, D.Sc. (Med.), Professor, Member of the Medical Association, no conflict of interest.

Larisa E. Golovanova^2,16, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Elena E. Savelieva¹¹, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Aleksandr O. Kuznetsov^1,4, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Aleksandr V. Pashkov¹⁵, D.Sc. (Med.), Member of the Union of Pediatricians of Russia, no conflict of interest.

Gaziz Sh. Tufatulin^2,16,17, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Denis V. Kovlen¹³, D.Sc. (Med.), Head of the Department of Physical and Rehabilitation Medicine, no conflict of interest.

Oksana N. Vladimirova¹⁸, D.Sc. (Med.), Member of the Medical Association, no conflict of interest.

Mikhail V. Sukhinin^3,6, Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Liya V. Toropchina⁵, Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Serafima B. Sugarova², Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Natalya A. Lebedeva¹², Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Ekaterina S. Garbaruk^9,18, Ph.D. (Biol.), Member of the Medical Association, no conflict of interest.

Elena A. Levina², Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Sergey V. Levin², Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Kirill L. Meshcheryakov⁷, Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Anna V. Petrova⁶, Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Vitaly L. Karpov^1,3, Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Evgenia R. Tsygankova^8,10, Ph.D. (Med.), Associate Professor, Member of the Medical Association, no conflict of interest.

Igor V. Goryainov^19,20, Ph.D. (Med.), Member of the Medical Association, no conflict of interest.

Irina V. Naumova¹⁵, Ph.D. (Med.), Member of the Union of Pediatricians of Russia, no conflict of interest.

Vera V. Zherenkova⁶, Member of the Medical Association, no conflict of interest.

Gyulnara R. Abuseva¹³, Lecturer at the Department of Physical and Rehabilitation Medicine, no conflict of interest.

Developing institutions:

1. National Medical Research Center for Otorhinolaryngology of the Federal Medical-Biological Agency of Russia.

2. Saint Petersburg Research Institute of Ear, Throat, Nose and Speech of the Russian Ministry of Health.

3. Russian Medical Academy of Continuous Professional Education of the Russian Ministry of Health, Department of Sensory Impairment Rehabilitation Technologies.

4. Pirogov Russian National Research Medical University of the Russian Ministry of Health.

5. Russian Medical Academy of Continuous Professional Education of the Russian Ministry of Health, Department of Pediatric Otorhinolaryngology.

6. Rehabilitation Center (for Hearing-Impaired Children) of the Russian Ministry of Health.

7. National Medical Research Center for Children's Health of the Russian Ministry of Health.

8. Russian Medical Academy of Continuous Professional Education of the Russian Ministry of Health, Department of Audiology.

9. First Saint Petersburg State Medical University named after Academician I.P. Pavlov, Laboratory of Speech and Hearing of the Research Center.

10. Sverжевsky Research Clinical Institute of Otorhinolaryngology of the Moscow City Health Department.

11. Bashkir State Medical University of the Russian Ministry of Health, Department of Otorhinolaryngology with a Course of DPE.

12. M.K. Ammosov North-Eastern Federal University.

13. Kirov Military Medical Academy of the Russian Ministry of Defense, Department of Physical and Rehabilitation Medicine.

14. National Medical Research Center for Rehabilitation and Balneology of the Russian Ministry of Health.

15. Research Institute of Pediatrics and Children's Health of the Scientific and Clinical Center No. 2 of the Petrovsky National Research Center of Surgery, Ministry of Science and Higher Education of the Russian Federation

16. Pavlov First Saint Petersburg State Medical University of the Ministry of Health of the Russian Federation

17. Saint Petersburg State Treasury Healthcare Institution "City Children's Audiology Center"

18. Saint Petersburg State Pediatric Medical University of the Ministry of Health of the Russian Federation

19. Saint Petersburg State Research Institute of Medical and Social Evaluation, Expertise and Rehabilitation of the Ministry of Labor of the Russian Federation

20. Main Bureau of Medical and Social Expertise in Saint Petersburg of the Ministry of Labor of the Russian Federation

21. I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

Appendix A2. Methodology for Developing Clinical Guidelines

The methodology for developing clinical guidelines is based on a comprehensive analysis of the current state of the problem, literature data, and regulatory legal acts, followed by discussion within the professional community and amendments based on the results of resolutions of conferences and national congresses, provided they are approved by the Ministry of Health.

Despite the common ICD-10 diagnosis code H90.3 in adults and children, the methodology for diagnosing and rehabilitating hearing impairments in children (especially early age) and adults differs cardinally; therefore, recommendations for the diagnosis of congenital hearing loss and the audiologic screening system, which seamlessly transitions into rehabilitation and dispensary follow-up, are presented separately.

Target audience of these clinical guidelines (in accordance with the Order of the Ministry of Health of the Russian Federation dated May 2, 2023, No. 205n "On Approval of the Nomenclature of Positions of Medical and Pharmaceutical Workers"):

1. Otorhinolaryngologist-audiologists and prosthetist-audiologists;

2. Otorhinolaryngologists;

3. Pediatricians, neonatologists, neurologists, general practitioners (family physicians).

Table 1. Scale for assessing levels of evidence (LE) for diagnostic methods (diagnostic interventions)

Table 2. Scale for assessing levels of evidence (LE) for prevention, treatment and rehabilitation methods (preventive, therapeutic, rehabilitation interventions)

Table 3. Scale for assessing grades of recommendation (GR) for prevention, diagnosis, treatment and rehabilitation methods (preventive, diagnostic, therapeutic, rehabilitation interventions)

Procedure for updating clinical guidelines.

The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

1. Procedure for the provision of medical care in the profile of "audiology and otorhinolaryngology": Order of the Ministry of Health of the Russian Federation dated April 9, 2015, No. 178n "On Approval of the Procedure for Providing Medical Care to the Population in the Profile of Audiology and Otorhinolaryngology"

2. Order dated September 17, 2014, No. 526n "On Approval of the Standard of Specialized Medical Care for Bilateral Sensorineural Hearing Loss after Cochlear Implantation in Patients Requiring Replacement of the Cochlear Implant Speech Processor" (as amended on December 22, 2014)

3. Order dated September 17, 2014, No. 527n "On Approval of the Standard of Specialized Medical Care for Bilateral Sensorineural Hearing Loss after Cochlear Implantation (Excluding Replacement of the Cochlear Implant Speech Processor)"

4. Order of the Ministry of Health of the Russian Federation dated October 23, 2019, No. 878n "On Approval of the Procedure for Organizing Medical Rehabilitation of Children"

5. Order of the Ministry of Labor and Social Protection of the Russian Federation and the Ministry of Health of the Russian Federation dated June 10, 2021, No. 402n/631n "On Approval of the List of Medical Examinations Necessary to Obtain Clinical and Functional Data Depending on the Disease for the Purpose of Conducting Medical and Social Expertise"

6. Order of the Ministry of Labor and Social Protection of the Russian Federation dated March 5, 2021, No. 107n "On Approval of the Terms of Use of Rehabilitation Technical Aids, Prostheses and Orthopedic Products"

7. Order of the Ministry of Labor of Russia dated August 27, 2019, No. 585n "On Classifications and Criteria Used in the Implementation of Medical and Social Expertise of Citizens by Federal State Institutions of Medical and Social Expertise".

8. Order of the Ministry of Labor of Russia dated February 13, 2018, No. 86n "On Approval of the Classification of Rehabilitation Technical Aids (Products) within the Framework of the Federal List of Rehabilitation Measures, Rehabilitation Technical Aids and Services Provided to a Disabled Person, Approved by the Order of the Government of the Russian Federation dated December 30, 2005, No. 2347-r".

9. Letter of the Ministry of Health and Social Development of the Russian Federation dated April 1, 2008, No. 2383-RKH "On Universal Audiological Screening of First-Year Children in Obstetric Facilities, Children's Polyclinics, and Hearing Rehabilitation Centers".

10. Letter of the Ministry of Health of Russia dated June 15, 2000, No. 2510/6642-32 "On the Introduction of Criteria for Patient Selection for Cochlear Implantation, Methods of Preoperative Examination, and Prediction of the Effectiveness of Rehabilitation in Implanted Patients". Order of the Ministry of Labor and Social Protection of the Russian Federation dated April 27, 2023, No. 342n "On Approval of the List of Indications and Contraindications for Providing Disabled Persons with Rehabilitation Technical Aids" (Registered with the Ministry of Justice of Russia on June 7, 2023, No. 73766). Decree of the Government of the Russian Federation dated April 5, 2022, No. 588 "On Recognition of a Person as Disabled (as amended on November 28, 2023)

The severity of identified persistent impairments of hearing functions and other functions is assessed as a percentage (Fig. 1).

Figure 1. Severity of persistent body function impairments (hearing function and other functions)

Disability is established in children in the presence of the following criteria:

1) Hearing impairment in a child due to bilateral sensorineural hearing loss:

- Grade II (41‒55 dB), in the presence of persistent moderate impairments of language and speech functions;

- Grade III (56‒70 dB), in the absence or presence of persistent impairments of language and speech and/or mental functions;

- Grade IV (71‒90 dB), including after unilateral or bilateral cochlear implantation;

- bilateral deafness (more than 91 dB), including after unilateral or bilateral cochlear implantation;

- combination of Grade III‒IV hearing loss (in one ear) and deafness (in the other);

- with combined vision and hearing impairment, as well as articulation (speech) and voice production disorders.

2) Presence of life activity restriction in the categories of ability to self-care, independent movement, orientation, communication, behavior control, learning, and employment. Disabled children with hearing impairment are characterized by restricted ability to communicate, orient, and learn of varying degrees of severity, determined in correlation with the age characteristics of the child.

3) Need for social protection measures, including rehabilitation.

A special form (f. 088/u) was used for referral to medical and social expertise (MSE), which was approved by a joint order of the Ministry of Labor and Social Protection of the Russian Federation and the Ministry of Health of the Russian Federation.

After receiving the results of the citizen's hearing function study, the medical organization providing preventive and therapeutic care (most often the district polyclinic) proceeds to draw up the referral for MSE.

Figure 2. Block diagram of the organization of hearing examination when referring children for MSE

*PEI – preschool educational institution

** PMAC – psychological, medical and pedagogical commission

Figure 3. Block diagram of the implementation of the program for additional examination of a child with hearing impairment

In accordance with the current edition of the Rules for Recognizing a Person as Disabled, in bilateral sensorineural hearing loss of grades III‒IV and deafness, disability is established without specifying the re-examination period. In conductive or mixed hearing loss, disability is established for 1 year, and after 4 years of follow-up, if life activity limitations cannot be compensated, without specifying the re-examination period.

For all citizens recognized as disabled, an individual rehabilitation and habilitation program (IRHP) of a disabled person (disabled child) is developed. It should be noted that:

- rehabilitation of disabled persons is a system and process of full or partial restoration of the abilities of disabled persons to everyday, social, professional, and other activities;

- habilitation is a system and process of formation of abilities that were absent in disabled persons for everyday, social, professional, and other activities.

Goals of rehabilitation or habilitation: elimination or the most complete possible compensation of activity limitations caused by a health disorder with a persistent dysfunction of the body, for the purpose of social adaptation of disabled persons, achievement of their financial independence, and their integration into society.

The individual rehabilitation and habilitation program (IPRA) is a set of rehabilitation measures optimal for a disabled person, including specific types, forms, scopes, timeframes, and procedures for implementing medical, vocational, and other rehabilitation measures aimed at restoring or compensating for impaired bodily functions, and developing, restoring, or compensating for the disabled person's ability to perform certain types of activities. The development of the IPRA is carried out by specialists of the bureau (main bureau, Federal bureau) taking into account the recommended medical rehabilitation or habilitation measures indicated in the referral for medical and social expertise issued by an organization providing medical care to citizens. The development of the IPRA regarding recommendations for the provision of medical rehabilitation equipment and rehabilitation or habilitation services provided to a disabled person (disabled child) at the expense of the federal budget is carried out based on the list of medical indications and contraindications for the provision of medical rehabilitation equipment to disabled persons.

The IPRA is developed for a period corresponding to the duration of the established disability group (the "disabled child" category). The period during which rehabilitation or habilitation measures are recommended must not exceed the validity period of the IPRA.

Federal state institutions of medical and social expertise send an extract from the IPRA to the relevant executive authorities, local self-government bodies, and organizations regardless of their legal forms and ownership types, which are entrusted with carrying out rehabilitation or habilitation measures provided for by the IPRA, by posting the relevant information in the federal state information system "Federal Register of Disabled Persons".

The executive state authority of a constituent entity of the Russian Federation in the relevant field of activity, the regional branch of the Social Fund of Russia, and in certain cases, the administration of residential-type institutions or institutions of the Federal Penitentiary Service, organize the work on the implementation of IPRA measures.

* SSEA – state executive authority

** FPS – Federal Penitentiary Service

Figure 4. Block diagram of the transfer of extracts from the IPRA of a disabled child

with hearing impairment to bodies and institutions

11. The executive state authority of a constituent entity of the Russian Federation in the relevant field of activity shall submit information to MSE institutions on the implementation of the rehabilitation or habilitation measures assigned to them in the disabled child's IPRA, in the form and manner approved by an order of the Ministry of Labor of Russia

Diagnostic examination protocol

Rating scales, questionnaires, and other patient assessment tools provided in clinical guidelines.

Parent Questionnaire "LittlEARS Early Speech Development Questionnaire" (LittlEARS)

Instructions for parents on completing the questionnaire

Please fill out the questionnaire.

1. All answers must be marked in the "Answer" column with "YES" or "NO".

- Mark "YES" if you have observed this reaction in the child at least once.

- Mark "NO" if you have never observed this reaction in the child or are unsure how to answer this question.

2. For children under 6 months of age, the questionnaire should be filled out up to question No. 14. For older children, the entire questionnaire should be filled out, as they may exhibit auditory reactions to various questions in the questionnaire.

To assess the dynamics of the child's auditory development, the questionnaire should be completed at 1-month intervals.

Child's full name ____________________________ Age _______ Date of birth _________

Date of completion __________ Diagnosis _____________________________________________

Unaided / with hearing aid / CI (Right/Left ear)

If using HA/CI, since what age has the HA/CI been worn

Count the number of "YES" answers _______ . Maximum score is 35.

Analysis of the questionnaire for parents

"Assessment of Auditory Behavior in Infants and Toddlers"

The concept of the questionnaire is that a child's auditory responses develop according to their age. In a child with hearing loss, hearing begins to develop after they are fitted with a HA/CI. From this moment, their "hearing age" begins to be counted.

Assessment of "hearing age":

1. In normal-hearing children, the "hearing age" corresponds to the child's chronological age.

2. In children with a CI (or HA), the "hearing age" corresponds to the duration of the period since the first mapping of the CI (or HA).

Table 1 presents the mean expected and minimum "hearing age" values for normal-hearing children aged from birth to 24 months.

After the parents complete the questionnaire, the child receives a total score, which is compared with the table data.

Table 1. Mean expected and minimum "hearing age" values for children (auditory development according to the child's age)

Meaning Integration Scale (MIS) Questionnaire

Child's full name _____________________________ Age _______ Date of birth ________

Date of 1st mapping of HA/ CI/ABI _________________ Date ____________

The questionnaire assesses the effectiveness of the child's use of a HA/CI during communication in daily situations based on observations by parents and educators working with the child. Mark the appropriate number for each question.

Rating (0-4): 0 = never; 1 = rarely (>25%); 2 = sometimes (>50%); 3 = frequently (>75%); 4 = always.

Mark the appropriate number for each question. For questions 1 and 2, both the main and sub-questions must be answered.

Total score:__________/ 40

1. Child's need score for wearing HA/CI (questions 1-2): __________

2. Score for child's spontaneous reactions to sounds (questions 3-6): _________

3. Score for recognition of environmental sounds (questions 7-10): ________

Speech Intelligibility Rating (SIR) Questionnaire

Child's full name ____________________________ Age _______ Date of birth _________

The questionnaire assesses the child's use of voice and speech with a CI/HA during communication in daily situations based on observations by parents and educators working with the child.

Rating (0-4): 0=never; 1=rarely (>25%, otherwise uses everyday gestures, sign language, fingerspelling); 2=sometimes (>50%, otherwise uses gestures, fingerspelling, drawings); 3=frequently (>75%); 4=always. For each question, indicate the appropriate number.

Total score:__________/ 40

The questions are divided into 3 groups (questions 4-10 are intended for children over 2 years of age):

1. Voice control score (questions 1-3): _______

2. Speech sound production score (questions 4-8): ________

3. Dominant communication strategy score (questions 9-10):_______

Modified scale

Categories of Auditory Performance (CAP)

Enhanced Speech Intelligibility Rating Scale for Hearing-Impaired Children

M-CHAT

Modified Checklist for Autism in Toddlers (M-CHAT) [53]

Instructions: Please rate how well the following statements characterize your child. Try to answer every question. If this behavior occurred rarely (you saw it only once or twice), mark it as uncharacteristic of the child.

M-CHAT Interpretation Instructions

The child fails the screening if there are two or more failed responses on critical items or three failed responses on any items. Yes/No answers are converted to pass/fail. Below are the responses corresponding to a "fail" code for each M-CHAT item. Bold uppercase text indicates critical items.

Not all children who fail the M-CHAT screen meet the diagnostic criteria for autism spectrum disorders. However, children who fail the screen require a comprehensive evaluation by a physician and a mandatory referral to a specialist for developmental assessment.

Appendix C. Patient Information

At stage I of the audiologic screening, if the test is failed, parents must be informed that the screening procedure is not a diagnosis, but merely a selection for the diagnosis of hearing impairments, which must be performed before 3 months of age. The test based on otoacoustic emission recording can be false-positive due to external and middle ear issues. If otoacoustic emissions are not recorded, the child should be referred to stage II of audiologic screening at an audiology office or center with the diagnosis of "unspecified hearing loss".

Example of an information leaflet "Newborn Audiologic Screening":

"For the first three years of life, a child learns to listen and speak. If a baby does not hear well, they will have difficulty understanding speech or may not be able to understand it at all. Because of this, it is hard to learn to speak. About 3 out of 1,000 babies are born with hearing loss, 1 of them with profound hearing loss. In past years, the world of sound and a fulfilling life would have been closed to such a child; they would have lacked speech communication and social adaptation. Today, new possibilities have emerged for the early diagnosis of hearing and auditory-speech development in children with hearing impairment. With a timely initiated support program, children with hearing loss differ very little from their peers. They attend regular kindergartens and schools, learn to play musical instruments, and study foreign languages. However, the support program is most effective if started in the first months of the baby's life. That is why all newborns must undergo a hearing check immediately after birth.

A quick, painless, and completely safe method for the baby's health is used to check hearing. Hearing is tested on days 3–4 using the evoked otoacoustic emissions recording method. The examination is performed during sleep or while the baby is resting. A small probe is inserted into the child's ear, through which sound is delivered. A healthy, hearing ear responds with an "echo" to this sound.

In the absence of an "echo", hearing loss must by no means be diagnosed immediately. The reasons for the absence of an "echo" may include: a) the presence of postpartum debris in the external auditory canal; b) middle ear diseases; c) true pathology of the auditory system.

If the "echo" is not recorded, your baby's screening should be repeated in 1 month.

Hearing does not always remain normal and may deteriorate over time. If you have doubts about whether your baby hears and understands speech well, consult a doctor immediately and have your child's hearing checked."

During the initial diagnosis of sensorineural hearing loss in children, parents will need to review a large amount of information regarding the medical, rehabilitative, social, and educational aspects of hearing loss. The test results must be interpreted for the parents, and a plan for necessary additional examination (if needed), treatment, and rehabilitation must be drawn up.

Treatment issues are of particular relevance and urgency in acute sensorineural hearing loss. From the perspective of evidence-based medicine, the lack of effective treatment for persistent sensorineural hearing loss requires the timely initiation of impaired auditory function correction.

In unilateral hearing loss, additional differential diagnostic measures are required.

According to indications, the patient is referred to medical and social expertise (MSE) to address the issue of assigning the status of a "disabled child", followed by drafting an individual rehabilitation or habilitation program (IRHP) and choosing the correction method (traditional hearing aids, implantable hearing aids, cochlear implantation). The parents of the child are informed about the procedure for providing high-tech medical care (HMC).

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

No