List of Abbreviations
HMT – high-tech medical care
dB HL – decibels hearing level
ISP – individual rehabilitation program
MSE – medical and social expertise
CI – cochlear implantation
ABR – auditory brainstem responses
OAE – otoacoustic emission
ASHL – acute sensorineural hearing loss
HA – hearing aids
SNHL – sensorineural hearing loss
InSCMP – inpatient specialized medical care
SISI / RRec – recruitment phenomenon
CSHL – chronic sensorineural hearing loss
Terms and Definitions
Pure-tone audiometry (audiometry) – a psychoacoustic method for examining hearing based on the patient's subjective perception of acoustic stimuli of various intensities.
Short-latency auditory brainstem responses (ABR) are indicators reflecting the electrophysiological activity of the auditory pathway structures, recorded from the scalp in response to acoustic stimulation. The test results are displayed as a complex of positive peaks (waves I–VII), of which the V peak is the most stable.
Cochlear implantation (CI) is a method of rehabilitating grade IV sensorineural hearing loss and deafness using a cochlear implant system, which includes an implantable part (an electrode array inserted into the cochlea and a receiver-stimulator placed subcutaneously in the retroauricular area) and an external part (a speech processor and a transmitting antenna).
Otoacoustic emission (OAE) consists of weak acoustic oscillations generated by the outer hair cells of the organ of Corti, which can be recorded by a special probe in the external auditory canal. OAE is not recorded if hearing thresholds exceed 25–30 dB HL in the frequency range from 1 kHz to 4 kHz, covering most of the speech frequency zone.
Sensorineural hearing loss (SNHL) is a form of hearing impairment (up to total loss) characterized by damage to any part of the sound-perceiving portion of the auditory analyzer, ranging from the sensory apparatus of the cochlea to neural structures.
Bilateral cochlear implantation is a complex of audiological, surdopedagogical, psychological, and surgical procedures for installing two cochlear implant systems in both ears.
Cochlear implant system is a device that provides direct electrical stimulation to surviving nerve fibers in the event of damage to (or absence of) the receptor apparatus of the cochlea (hair cells).
Cochlear implant is the part of the cochlear implant system that transmits sound information—converted into electrical impulses by the speech processor—directly to the auditory nerve fibers, bypassing the damaged receptor apparatus of the cochlea.
Speech processor is the part of the cochlear implant system designed to capture ambient sound information, perform analysis and coding, and subsequently transmit the digital signal to the cochlear implant.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Sensorineural hearing loss (SNHL) is a form of hearing impairment (up to total loss) characterized by damage to any part of the sound-perceiving portion of the auditory analyzer, ranging from the direct sensory apparatus of the cochlea to neural structures [1, 2].
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
The pathomorphological substrate of SNHL is a quantitative deficit of functioning neural elements at various levels of the auditory analyzer, from the peripheral section—the spiral organ—to the central division represented by the auditory cortex of the temporal lobe of the brain. Damage to receptors—the sensory structures of the cochlea—is the primary morphofunctional condition for the development of SNHL up to complete deafness. The initial pathomorphological substrate in the cochlea is a dystrophic process in the hair cells, which can be reversible if medical care is initiated in a timely manner.
Significant etiological factors for the development of sudden and acute SNHL include:
o Infectious diseases (viral: influenza, mumps, measles, tick-borne encephalitis; bacterial: epidemic cerebrospinal meningitis, scarlet fever, diphtheria, typhus, syphilis).
o Toxic exposures (acute intoxications, including domestic and industrial; iatrogenic drug-induced damage by ototoxic drugs: aminoglycosides, loop diuretics, antineoplastic agents, nonsteroidal anti-inflammatory drugs, etc.).
o Circulatory system diseases (cardiovascular: hypertension, coronary artery disease; cerebral circulation disorders predominantly in the vertebrobasilar basin, alterations in blood hemorheology, etc.).
o Degenerative-dystrophic diseases of the spine (uncovertebral arthrosis of C1–C4, spondylosis, spondylolisthesis with the clinical presentation of "vertebral artery syndrome").
o Genetic monogenic disorders and genetic predisposition to adverse environmental factors.
1.3 Epidemiology of the Disease or Condition
About 6% of the global population (466 million people) have socially significant (greater than 40 dB) hearing loss of various etiologies. According to World Health Organization projections, in the absence of necessary care, the number of individuals with hearing loss will reach 630 million by 2030 and 900 million by 2050. According to WHO estimates, the number of patients with socially significant hearing loss in the Russian Federation exceeds 13 million people [3].
According to the Russian Ministry of Health, based on medical institution statistical reporting on healthcare utilization, the prevalence of bilateral sensorineural hearing loss among the adult population aged 18 years and older in 2017 was 490 per 100,000 of the corresponding age group, and among those above working age—1,094 per 100,000 [4].
1.4 Features of Coding the Disease or Condition
H90.3 – Bilateral sensorineural hearing loss;
H90.4 – Sensorineural hearing loss, unilateral with normal hearing on the opposite ear;
H90.5 – Unspecified sensorineural hearing loss;
H91.1 – Presbycusis;
H91.2 – Sudden idiopathic hearing loss;
H91.8 – Other specified hearing loss.
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;
acquired.
3. By course:
stable;
progressive;
fluctuating;
reversible.
4. By acuteness of the disease:
sudden – hearing loss develops within up to 12 hours;
acute – hearing loss develops within 1–3 days and persists for up to 1 month;
subacute – hearing loss persists for 1–3 months;
chronic (persistent) – hearing loss persists for more than 3 months.
5. By side affected:
unilateral;
bilateral (symmetric and asymmetric).
6. By etiology:
genetic (hereditary);
multifactorial (with hereditary predisposition);
acquired.
1.6 Clinical presentation of the disease or condition
The main signs of sensorineural hearing loss in adults are hearing loss, impaired speech intelligibility, tinnitus, and a sensation of a plugged ear.
When taking the medical history, special attention should be paid to the hearing status of close relatives, the nature of employment, the presence of infectious and somatic diseases, and regular medical follow-up (dispensary 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
Diagnostic criteria: the diagnosis is established based on complaints (hearing loss, tinnitus, impaired speech intelligibility), physical examination (otoscopic findings are normal), audiological test methods: tympanometry type A, acoustic reflexes present or absent depending on the degree of hearing loss, pure-tone audiometry: average air-conduction hearing threshold at frequencies of 500, 1000, 2000, and 4000 Hz $\ge$ 26 dB, air-bone gap $\le$ 10 dB, speech audiometry results consistent with pure-tone audiometry.
2.1 Complaints and Anamnesis
The complaints specify the onset time of hearing impairment, rate of progression, symmetry of hearing on the right and left, degree of communicative difficulties, and severity. Questions are actively asked about the presence of tinnitus in the patient, its severity and pitch, vestibular disorders, neurological symptoms, and other signs important for establishing a clinical diagnosis.
Characteristic signs of acute sensorineural hearing loss include:
o sudden unilateral or bilateral decrease in hearing (deterioration of speech intelligibility and sound perception) up to deafness;
o in some cases (acute trauma, labyrinthine artery blood flow impairment, toxic effects on labyrinthine structures), hearing loss may be accompanied by subjective tinnitus of varying pitch, sometimes acute vestibular and autonomic dysfunction presenting as ataxia, dizziness, nausea, diaphoresis, tachycardia, blood pressure fluctuations, and spontaneous nystagmus.
Among patient complaints, hearing impairment ranks first, combined with tinnitus, most frequently constant and predominantly of mixed pitch.
It is recommended to pay attention to the following risk factors: relatives with hearing loss (especially with matching degree, type of hearing loss, and onset conditions), employment in high occupational noise environments, use of ototoxic drugs, infectious diseases (meningitis, mumps, measles, influenza, and other infections), and somatic diseases [2, 5–8].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Complaints and anamnesic data allow clarifying the nature of hearing impairment and drawing up an individual differential diagnosis plan.
In case of acute sensorineural hearing loss, it is recommended to ask questions according to the following plan:
The following questions are recommended:
How are you feeling?
What is your body temperature?
Is there hearing loss or not, and if so, in which ear?
When and how did the hearing deterioration occur?
Was there a dynamic change (deterioration, improvement) in hearing?
What is the hearing loss associated with?
Was hearing impaired previously?
Is there tinnitus in one or both ears?
Was the hearing loss accompanied by dizziness?
What is the nature of the dizziness: can the patient indicate the direction in which the dizziness is directed, or is everything spinning chaotically without a clear direction?
Are there autonomic disorders (nausea, vomiting, diarrhea, sweating)?
If there was vomiting, did the dizziness and tinnitus decrease?
Has the patient previously noted episodes of systemic vertigo?
Has a diagnosis of Ménière's disease (syndrome) been established?
Does the patient suffer from otitis media?
Has the patient taken any medications?
What are the blood pressure values?
Does the patient suffer from hypertension, ischemic heart disease, or cerebrovascular disease?
Did head or cervical spine trauma precede the hearing loss?
Was the patient exposed to intense noise?
Is the sudden hearing deterioration related to swimming or taking a bath?
Is there any limb weakness, facial asymmetry, sensory disturbance, movement disorder, or visual impairment?
Was there dental treatment or dental prosthetics?
2.2 Physical Examination
It is recommended in patients with sensorineural hearing loss to ensure the absence of pathological changes in the structures of the external and middle ear. The otoscopic picture in sensorineural hearing loss corresponds to normal [1, 9, 74].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: patients with previous or existing middle ear pathology may also develop acute sensorineural hearing loss requiring appropriate treatment, so otoscopy is necessary but not sufficient for diagnosis and determination of management tactics.
2.3 Laboratory Diagnostics
It is recommended for all patients with sensorineural hearing loss to undergo a complete blood count (CBC) to assess the general condition of the body [2, 7].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
It is recommended for patients with sensorineural hearing loss in the presence of appropriate pathology to perform a blood lipid profile (cholesterol, LDL, HDL), general therapeutic biochemical blood test (glucose level, total and direct bilirubin, ALT, AST, creatinine, urea), coagulography (screening test of the hemostasis system) (aPTT, INR, prothrombin time, fibrinogen), and measurement of blood thyroid-stimulating hormone (TSH) level [2, 7].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
2.4 Instrumental Diagnostics
It is recommended for all patients with suspected sensorineural hearing loss to compile an auditory profile—testing hearing with whispered and conversational speech, and examining the hearing organ using tuning forks [1, 3, 4, 7, 9, 14, 15, 21].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: akumetric tests have a screening value, which is why they are sufficient at an ENT appointment for subsequent referral to an audiologist-otorhinolaryngologist.
It is recommended for all patients with suspected sensorineural hearing loss to determine hearing thresholds for air-conducted (125–8000 Hz) and bone-conducted tonal stimuli (250–6000 Hz), including in an expanded frequency range (125–20000 Hz) using pure-tone audiometry when normative values are met but there are complaints of hearing loss or subjective tinnitus. When diagnosing sudden and acute idiopathic hearing loss, pure-tone audiometry must be performed no later than 24 hours from the initial presentation. This is the baseline method for both primary diagnosis and follow-up [1, 7, 15]. During treatment, prior to patient discharge, pure-tone audiometry should be repeated to verify the restoration of hearing thresholds to normal or to determine the effectiveness of the treatment and further management tactics if normalization is not achieved or a stabilized state is reached (absence of further progression of hearing loss) [1, 7, 15]. In cases of diagnosed chronic bilateral sensorineural hearing loss, determine indications for correction of impaired auditory function using various methods (traditional hearing aids, implantable hearing aids, including cochlear implantation) to compensate for hearing loss. If necessary, determine indications for referring the patient for medical and social expertise and drawing up an individual rehabilitation program. If the patient is a user of an auditory technical rehabilitation device (hearing aid, bone conduction device, cochlear implant), evaluate the effectiveness of hearing aid fitting and perform adjustments if necessary [1, 7, 15] to determine the effectiveness of prosthetic hearing rehabilitation and compensation for hearing loss.
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: in the presence of asymmetric hearing loss, masking of the non-test (better-hearing) ear is performed.
It is recommended for all patients with suspected sensorineural hearing loss to perform suprathreshold tests (determination of discomfort thresholds, SISI, Lüscher test) to assess loudness recruitment abnormalities [1, 7, 15].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: The examination can be supplemented by comparing hearing thresholds with acoustic reflex thresholds at frequencies of 500–4000 Hz.
It is recommended for all patients with suspected sensorineural hearing loss to perform impedance audiometry (tympanometry and acoustic reflexometry on ipsilateral and contralateral sides) to exclude middle and inner ear pathology, differentiate from conductive hearing loss, and diagnose lesions of the auditory and facial nerves, central auditory pathways, and VII and VIII cranial nerve nuclei at the level of the medulla oblongata and pons [1, 8, 12, 15, 17].
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: when using reflex recording in a wide time window (up to 10 s), it is possible to determine acoustic reflex decay, indicating the presence of a retrocochlear lesion.
It is recommended for all patients with suspected sensorineural hearing loss to perform speech audiometry in quiet and in the presence of masking noise [3, 4, 9].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: provides information on speech perception and intelligibility thresholds; detection of speech-tonal dissociation may be a manifestation of central disorders.
It is recommended for all patients with suspected sensorineural hearing loss to perform electrocochleography for early detection of signs of endolymphatic hydrops [2, 14, 15].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: extratympanic electrocochleography is performed on an outpatient basis using an ear electrode and recording potentials to stimuli with initial rarefaction and condensation phases.
It is recommended for all patients with suspected sensorineural hearing loss to perform auditory brainstem response (ABR) testing, as well as transient-evoked otoacoustic emission (TEOAE) and distortion-product otoacoustic emission (DPOAE) testing for differential and topical diagnosis [1, 6, 9, 15].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: in asymmetric hearing loss, it is advisable to use low stimulus presentation frequencies and compare the V-I interpeak intervals on both sides.
It is recommended for all patients with suspected sensorineural hearing loss to perform vestibulometry to assess the condition of the vestibule and semicircular canals of the inner ear, as well as to detect labyrinth dysfunction and for differential diagnosis [1, 2, 15, 28].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
It is recommended for all patients with suspected sensorineural hearing loss to evaluate anatomical structures by performing brain magnetic resonance imaging (MRI), including with intravenous contrast (areas of the internal auditory canals, cerebellopontine angles, posterior cranial fossa), and computed tomography (CT) of the temporal bone, including with intravenous bolus contrast (high-resolution of the cochlea and internal auditory canals); these methods are of particular diagnostic value in unilateral or asymmetric hearing loss, but their performance is important regardless of the degree and symmetry of hearing loss [1, 2, 8-9, 14, 16, 18].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
It is recommended for all patients with suspected sensorineural hearing loss to evaluate anatomical structures by performing cervical spine radiography with functional tests, brain MRI, and in unilateral hearing loss—with contrast enhancement to verify acoustic neuroma [1, 2, 8-9, 14, 16, 18].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: MRI provides clarification of the differential and topical diagnosis, helps to clarify the level of the auditory analyzer lesion, and reveals anatomical features of ear structures [1, 2, 8-9, 14, 16, 18].
It is recommended for all patients with sensorineural hearing loss in the presence of corresponding diseases to undergo duplex scanning of brachiocephalic arteries with color Doppler flow mapping, duplex scanning of extracranial segments of brachiocephalic arteries, duplex scanning of intracranial segments of brachiocephalic arteries, and vein triplex scanning [2, 7].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
2.5 Other Procedures
It is recommended to have an appointment (examination, consultation) with a general practitioner, an appointment (examination, consultation) with a neurologist, and an appointment (examination, consultation) with an endocrinologist for various types of sensorineural hearing loss regardless of the disease duration. For special indications (presence of autoimmune diseases and immunodeficiency states), an immunological status assessment is required in case of combined immunodeficiency [1, 2, 8-9, 12, 14, 16, 18, 21].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: identification of lipid and carbohydrate metabolism disorders allows for a pathogenetically substantiated comprehensive treatment regimen.
During differential diagnosis, it is necessary to consider other forms of diseases manifesting as hearing loss, such as labyrinthopathies (resulting from chronic suppurative otitis media, previous radical middle ear surgery, or prior labyrinthitis), inner ear damage due to infectious diseases, intoxication, vestibular schwannoma (acoustic neuroma), cerebrovascular disorders in the vertebrobasilar system, multiple sclerosis, space-occupying brain lesions, traumatic brain and spinal injuries, cerumen impaction, diabetes mellitus, hypothyroidism, chronic kidney disease, etc.
The diagnosis of sudden sensorineural hearing loss at the pre-hospital stage is presumptive. Given the particular social significance of hearing for an individual, establishing this diagnosis, even tentatively, dictates a specific strategy—emergency cytoprotection of the inner ear sensory structures and pathways, and urgent medical evacuation to a multidisciplinary hospital (with an ENT department) for differential diagnosis, prescription, or adjustment of therapy.
Typical for a peripheral level lesion (cochlear hearing loss) is a downward-sloping audiogram in the absence of an air-bone gap, along with the presence of recruitment (abnormal loudness adaptation/recruitment phenomenon). In central cortical and subcortical forms of hearing loss, a downward-sloping audiogram in the absence of an air-bone gap is also more frequently detected, but without recruitment. Speech intelligibility in noise and sound localization function are significantly impaired.
Following an MRI and/or CT scan of the brain, a consultation with a neurosurgeon is recommended if a vestibular schwannoma or another space-occupying lesion is suspected [1, 2, 8-9, 14, 16, 18].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
Treatment for sudden and acute sensorineural hearing loss:
With a diagnosis of "Acute sensorineural hearing loss," the patient is referred for treatment in an inpatient or day care hospital setting.
Emergency hospitalization in the otolaryngology (or neurology) department is recommended [2, 8-9, 21].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
It is recommended to follow a hearing-protective regimen [2, 8-9, 21].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
It is recommended to administer intravenous infusion therapy: glucocorticoids in a tapering schedule (e.g., #dexamethasone** [27] in 100 ml of sodium chloride** on days 1 and 2 – 24 mg, days 3 and 4 – 16 mg, day 5 – 8 mg, day 6 – 4 mg, day 7 – no drug administered, day 8 – 4 mg) (prescribed individually, taking into account all the patient's comorbid conditions, contraindications to medication, and potential adverse effects) [27].
Level of Recommendations CLevel of Evidence 3Level of Recommendation C (Level of Evidence – 3)
Comment: both systemic glucocorticoid administration (oral or infusion) and local administration (transtympanic and transtubular) are possible, and they can be combined. In the acute period, combining different routes of steroid administration allows for lower systemic doses without reducing overall efficacy.
It is recommended for a suspected ischemic origin of acute sensorineural hearing loss (against the background of cardiovascular disorders) to administer intravenous infusion therapy with other drugs for nervous system disorders (e.g., #ethylmethylhydroxypyridine succinate** 5% – 4 ml in 16 ml of sodium chloride** for 8–10 days) [28].
LeRR CLoE 4Level of recommendation confidence C (level of evidence – 4)
Treatment for chronic sensorineural hearing loss:
It is recommended to conduct maintenance treatment courses according to individual indications, taking into account the patient's somatic pathology [2, 8-9, 21].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
It is recommended to follow a hearing-protective regimen [2, 8-9, 21].
LeRR CLoE 4Level of recommendation confidence C (level of evidence – 4)
It is recommended to treat underlying somatic conditions and administer courses of maintenance therapy 1–2 times a year using oral medications that improve cerebral and labyrinthine blood flow, as well as tissue and cellular metabolism processes [2, 8-9, 21].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comment: in certain forms of chronic sensorineural hearing loss (e.g., hereditary, post-meningitic, or ototoxic), long-term therapeutic interventions are ineffective [2, 8-9, 21].
3.2 Surgical Treatment
Implantable hearing aids and cochlear implantation are described in the section "Medical rehabilitation, medical indications, and contraindications for rehabilitation methods."
4. Medical Rehabilitation and Sanatorium-Resort Treatment
1. It is recommended to select and fit hearing aids for patients with chronic sensorineural hearing loss when average hearing thresholds in the speech frequency range (500, 1000, 2000, and 4000 Hz) in the better-hearing ear are ≥31 dB [8, 9, 16, 19, 22].
GRS CLOE 5Grade of Recommendation Strength C (Level of Evidence – 5)
2. It is recommended to install a middle ear implant in patients with chronic sensorineural hearing loss when hearing aids cannot be used, provided that bone conduction thresholds at 500 Hz do not exceed 55 dB and at high frequencies do not exceed 75 dB [2, 8-9, 21].
URC CLEL 5Level of Recommendation C (Level of Evidence – 5).
3. It is recommended to perform auditory-verbal rehabilitation via cochlear implantation in patients with bilateral grade IV sensorineural hearing loss or deafness [2, 8-9, 21].
GRS CLOE 5Grade of Recommendation Strength C (Level of Evidence – 5)
4. It is recommended to administer electrical stimulation to patients with sensorineural hearing loss and tinnitus [30, 31, 32, 33, 34, 35, 36].
GCR BLEL 2Grade of Recommendations B (Level of Evidence – 2)
Comments: continuous direct current in pulse mode, rectangular pulses, and fluctuating currents are used. Current intensity ranges from 0.5 to 2 mA. The stimulation frequency is adjusted depending on the tinnitus frequency. The procedure lasts 10–15 minutes, with a course of 10–15 procedures. The procedure is contraindicated in patients using a cochlear implant system, auditory brainstem implant, or middle ear implant.
5. It is recommended to perform a course of hyperbaric oxygen therapy for ear diseases from 10 to 20 procedures at a pressure of 1.5–2.5 atm, lasting from 1 to 2 hours in patients with sensorineural hearing loss [37, 38, 39, 40, 55, 56].
GCR ALEL 1Grade of Recommendations A (Level of Evidence – 1)
Comments: therapy is optimally initiated within 1 month from the onset of the disease. The best results are achieved when combined with glucocorticoid intake.
6. It is recommended to include reflexology procedures for auditory organ disorders in the treatment course of patients with sensorineural hearing loss [41, 42, 43, 44, 45, 46, 47, 48, 55, 56].
GCR ALEL 1Grade of Recommendations A (Level of Evidence – 1)
Comments: reflexology is effective as an adjunctive therapy for sensorineural hearing loss, including in the late stages of the disease. Electroacupuncture is more effective than classical acupuncture. During electroacupuncture, electrical impulses (1 ms) of alternating polarity with a frequency of 67 Hz are applied. The amplitude is adjusted to produce a sensation of mild tingling. Electrodes are placed on each pair of acupuncture points for 10 minutes. A course consists of 10–15 procedures, daily or every other day.
7. It is recommended to perform transcranial electrical stimulation of the brain [49, 50, 51, 52, 53, 54, 55, 56].
Level of Evidence AGrade of Evidence 2Level of Recommendations A (Grade of Evidence – 2)
Comments: The greatest therapeutic effect is observed in patients with initial and mild degrees of hearing loss, including the use of audio-selective transcranial electrical stimulation technology. Current intensity is up to 1.0 mA. The duration of each procedure is 20 minutes. A course of 15 transcranial electrical stimulation sessions is administered every other day. The procedure is contraindicated in patients using a cochlear implant system, auditory brainstem implant, or middle ear implant.
8. It is recommended to include metered physical exercise in the treatment course of patients with sensorineural hearing loss [63].
Leveled of Evidence CLevel of Data Reliability 2Level of evidence C (level of data reliability – 2)
Comments: balance training and breathing exercises are performed. Physical therapy sessions are conducted in courses for 3 months, 3 times a week for 40 minutes. Additionally, terrain cure (terrenkur) for at least 20 minutes daily is recommended.
9. It is recommended to perform transcranial magnetic stimulation, including in patients with tinnitus associated with sensorineural hearing loss [53, 55, 56].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: repetitive transcranial magnetic stimulation is recommended, including stimulation of the temporoparietal, frontal, and prefrontal cortical areas. The procedure is contraindicated in patients using a cochlear implant system, auditory brainstem implant, or middle ear implant.
10. It is recommended to prescribe medicinal ultraphonophoresis and aural drug electrophoresis for auditory organ diseases in patients with sensorineural hearing loss [55, 56, 57].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: 1.5–2.0 ml of a medicinal substance warmed to 36–37 °C is instilled into the external auditory canal. The ultrasound transducer is placed in the scapha of the auricle, and the active electrode is positioned on the mastoid process of the opposite ear. The duration of the procedure is 5–15 minutes at a frequency of 880 kHz and a power of up to 0.2 W/cm2 in continuous mode, with a galvanic current of up to 0.3 mA. A course consists of 10–12 procedures. The procedure is contraindicated in patients using a cochlear implant system, brainstem implant, or middle ear implant.
11. It is recommended to include medical massage procedures in the treatment course for patients with sensorineural hearing loss [73].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: therapeutic massage (classical, acupressure, vacuum) is performed: medical neck massage, collar zone massage, daily, up to 10 procedures per course.
12. It is recommended to perform laser therapy as part of the complex therapy for sensorineural hearing loss [55, 56, 59, 60].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: performed either as low-level laser therapy (intravenous laser blood irradiation) combined with pentoxifylline administration, or via an endaural technique. For intravenous laser blood irradiation (ILBI), the laser wavelength is 630 nm, the power at the fiber end is 1.0–2.0 mW, the procedure duration is 25–30 minutes, with a course of 5–7 procedures daily. The endaural technique uses a wavelength of 808–810 nm, an energy flux density of 8–15 mW/cm2, a procedure duration of 8–10 minutes, and a course of 12–14 procedures daily.
13. It is recommended to include auditory training using virtual reality technologies in the treatment regimen for patients with sensorineural hearing loss [61, 62].
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: auditory training is recommended as an adjunctive treatment method in individuals with mild chronic sensorineural hearing loss and in individuals with hidden hearing loss. Auditory training is conducted on the basis of virtual reality using various audiovisual scenarios in a virtual reality headset. Sessions are held daily, one procedure per day for 7 days, with the duration of each session being 15 minutes with a single 2-minute break.
14. It is recommended to conduct vestibular training, including the use of virtual reality technologies, in patients with sensorineural hearing loss [62, 63, 64, 65, 66, 67, 68].
GCR BLoE 3Level of Evidence B (Level of Confidence of Evidence – 3)
Comments: a course of metered physical loads is recommended for training balance and equilibrium, including the use of virtual reality, daily or every other day, for 30 minutes, with a course of 20 procedures.
15. It is recommended to use specialized mobile smartphone applications with real-time noise reduction to improve auditory function in individuals with sensorineural hearing loss, including those after cochlear implantation [55, 69, 70, 71].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Items 4–15 are most commonly used during sanatorium-resort treatment after a doctor's consultation.
№ | ICD-10 code | Disease name | Form, stage, phase, severity degree diseases | Resorts, sanatorium-resort organizations |
|---|---|---|---|---|
1 | H90.3 | Bilateral sensorineural hearing loss | In the chronic stage. By time of onset: congenital or acquired. 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. | Sanatorium-resort organizations. Resorts: 1) balneological resorts with carbon dioxide, radon, hydrogen sulfide, sodium chloride, iodine-bromine waters; 2) climatic resorts: seaside, forest, lowland, low- and mid-mountain mud-bath |
2 | H90.4 | Unilateral sensorineural hearing loss with normal hearing in the contralateral ear | ||
3 | H90.5 | Sensorineural hearing loss, unspecified |
In accordance with Federal Law No. 323-FZ dated November 21, 2011, "On the Fundamentals of Protecting Citizens' Health in the Russian Federation," the purpose of sanatorium-resort treatment for individuals with hearing impairments is the complete or partial restoration of impaired functions and/or compensation for lost functions of the affected organ or body system, the maintenance of body functions during the completion of an acute pathological process or an exacerbation of a chronic pathological process, as well as the prevention, early diagnosis, and correction of potential functional disorders of damaged organs or 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.
Currently, the only effective method of rehabilitation for patients suffering from severe sensorineural hearing loss and deafness is cochlear implantation, which is a set of audiologic, deaf-educational (surdopedagogical), psychological, and surgical measures aimed at the complete or partial restoration of impaired functions and/or compensation for lost functions of the peripheral part of the auditory analyzer (the cochlea), the maintenance or development of auditory-speech function, 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 [8, 9, 13, 16, 18, 21].
The volume of this type of medical care provided to the adult population of the Russian Federation in a medical institution should not exceed 20% of the total volume of the state assignment for this type of high-tech medical care (HMC) [8, 9, 13, 16, 18, 21].
The warranty period for the internal part of the cochlear implant system (the implant) must be at least 10 years, and for the external part (the speech processor) at least 5 years [8, 9, 13, 16, 18, 21].
Indications for cochlear implantation:
1. Chronic bilateral sensorineural hearing loss, grade IV, with hearing thresholds of 80 dB or more according to auditory brainstem response (ABR) data.
2. Chronic bilateral sensorineural hearing loss, grade IV, with average hearing thresholds at core frequencies (500, 1000, 2000, 4000 Hz) of 80 dB or more according to pure-tone audiometry data.
3. Auditory perception thresholds in a free sound field using optimally fitted hearing aids (binaural hearing aid fitting) exceeding 55 dB at frequencies of 2–4 kHz.
4. Lack of marked improvement in speech perception from the use of optimally fitted hearing aids in severe bilateral sensorineural hearing loss (mean threshold greater than 95 dB) after at least 3–6 months of hearing aid use, according to the conclusion of the deaf-educational (surdopedagogical) commission.
5. The result of free-field speech audiometry using optimally fitted hearing aids in severe bilateral sensorineural hearing loss, after at least 3–6 months of hearing aid use, according to the conclusion of the deaf-educational (surdopedagogical) commission, is less than 40%.
Absolute contraindications for cochlear implantation:
1. Complete aplasia of the inner ear (Michel anomaly).
2. Complete cochlear ossification/obliteration.
3. Presence of severe somatic, neurological, and mental illnesses.
4. Intellectual deficiency and lack of communication capabilities (exceptions may be made for visually impaired and blind individuals).
Relative contraindications for cochlear implantation:
1. Lack of motivation and socio-psychological capacity for a long-term rehabilitation process in the patient (parents, legal representative, or proxy).
2. Partial cochlear obliteration (as determined by the medical commission of the center performing the surgical treatment).
3. Pronounced developmental anomalies of the cochlea and auditory nerve (as determined by the medical commission of the center performing the surgical treatment).
4. Acute or chronic inflammatory diseases of the ENT organs in the acute stage.
5. Retrocochlear pathology (as determined by the medical commission of the center performing the surgical treatment).
Binaural cochlear implantation
Bilateral (binaural) cochlear implantation is a set of audiologic, deaf-educational (surdopedagogical), psychological, and surgical procedures for placing two cochlear implant systems in both ears.
Bilateral (binaural) cochlear implantation is performed simultaneously—during a single surgical operation—or sequentially—during two separate surgical operations.
Bilateral (binaural) cochlear implantation provides:
bilateral stimulation of auditory pathways and auditory centers;
the ability to localize sound, i.e., "spatial" sound perception;
improved speech intelligibility in noise and when communicating with multiple speakers;
better dynamics of auditory and speech development, as speech sounds more natural, the process of sound perception requires less effort and is therefore less fatiguing;
preservation of sound perception function in the event of failure of one implant.
Indications for bilateral (binaural) cochlear implantation:
I. Indications for simultaneous bilateral (binaural) cochlear implantation: chronic bilateral sensorineural hearing loss, grade IV, and deafness following meningitis (with a provided hospital discharge summary) and as determined by the medical commission of the center performing the surgical treatment.
II. Indications for sequential bilateral (binaural) cochlear implantation:
1. Chronic bilateral sensorineural hearing loss, grade IV, and deafness in the presence of radiological signs of cochlear ossification/obliteration resulting from temporal bone fractures and otosclerosis, as determined by the medical commission of the center performing the surgical treatment.
2. Combined or isolated inner ear developmental anomalies, as determined by the medical commission of the center performing the surgical treatment.
In the aforementioned inner ear anomalies, the number of spiral ganglion cells is reduced, and unilateral cochlear implantation may be insufficient to achieve a satisfactory outcome in auditory-speech rehabilitation.
3. Presence of positive dynamics in hearing and speech development, successful social adaptation, and actively pursued psycho-pedagogical rehabilitation after unilateral cochlear implantation, provided there is high motivation from patients and their parents (legal representatives, trusted proxies) based on the conclusion of the medical commission of the center performing surgical treatment.
At the same time, the volume of sequential binaural cochlear implantations performed should not exceed 10% of the annual volume of this type of medical care provided at the institution.
Contraindications to bilateral (binaural) cochlear implantation:
These are similar to the general (absolute and relative) contraindications for cochlear implantation, with an interval of more than 5 years of using a unilateral cochlear implant system.
Revision cochlear implantation (reimplantation)
Indications for reimplantation:
Cause | Treatment methods |
|---|---|
Technical malfunction of the cochlear implant | Cochlear implant reimplantation |
Purulent inflammatory diseases of the middle ear on the implanted side (chronic suppurative otitis media, mastoiditis) | Sanation of the middle ear cavities, tympanoplasty. If preserving the cochlear implant is impossible, its explantation is performed while retaining the electrode array in the cochlea, with simultaneous contralateral cochlear implantation in the absence of contraindications. |
Extrusion of the internal body of the cochlear implant | Explantation with retention of the electrode array in the cochlea and simultaneous contralateral cochlear implantation in the absence of contraindications. |
Migration of the internal package of the cochlear implant making it impossible to operate the speech processor, extracochlear placement of the electrode array. |
Revision surgical treatment (reimplantation) is performed using a matching cochlear implant system (from the same manufacturer) due to the varying technical characteristics of implants. Performing a reimplementation using a cochlear implant system from a different manufacturer is possible only at the expense of extra-budgetary funds, which must be documented in the patient's (legal representative's) informed consent.
In the event of failure of the internal part of the cochlear implant system (implant) within the warranty period, the manufacturer, upon application by the medical facility where the patient sought diagnostics, conducts a preliminary expert evaluation and issues an initial report within 10 days with recommendations, including the need for removal of the internal part of the cochlear implant system (explantation), and provides the medical facility with the internal part of the cochlear implant system for revision surgery within 30 days from the issuance of the initial report.
The final determination of the malfunction of the internal part of the cochlear implant system is made following a technical expert evaluation of the extracted implant, performed by the manufacturer or an independent expert at the applicant's expense. The examination period must not exceed three months, after which the manufacturer sends a written final report to the medical facility regarding the condition of the internal part of the cochlear implant system, containing information on the potential causes that led to the cochlear implant failure.
5. Prevention and Dispensary Observation
It is recommended that patients with sensorineural hearing loss undergo follow-up dispensary visits (examination, consultation) by an otolaryngologist-audiologist at the audiology department/center at their place of residence to evaluate treatment efficacy and monitor the patient's condition [74, 12].
GRS CLOE 5Grade of Recommendation Strength C (Level of Evidence – 5)
Comments:
Evaluation of hearing aid fitting efficacy:
o Free-field speech audiometry in quiet and in noise.
o Questionnaires assessing quality of life.
Follow-up audiological testing and monitoring include:
1. Pure-tone audiometry – 1–2 times a year.
2. Speech audiometry in quiet and in noise – 1–2 times a year.
3. Adjustment of hearing aid amplitude-frequency amplification characteristics (based on audiological examination results) – 1–2 times a year.
In the event of acute deterioration or rapid progression of hearing loss, a repeat examination is scheduled immediately (to enable timely treatment with a good outcome for restoring hearing thresholds).
Evaluation of cochlear implantation efficacy:
Free-field speech audiometry in quiet and in noise after activation of the speech processor and during subsequent fitting sessions (2–4 times a year).
Questionnaires assessing quality of life.
Follow-up audiological testing and monitoring:
1. Pure-tone audiometry – 1–2 times a year.
2. Speech audiometry in quiet and in noise – 1–2 times a year.
3. Speech processor programming – 2–4 times a year.
6. Organization of Medical Care
Indications for hospital admission to a medical organization:
1) acute sensorineural hearing loss;
2) replacement of the cochlear implant speech processor (in inpatient or day-care settings);
Indications for discharging a patient from a medical organization:
1) recovery;
2) improvement.
7. Additional Information
Hearing loss has significant social, medical, and psychological implications. Uncompensated hearing loss leads to a reduced quality of life, a lower level of educational potential realization, and limits career choices in proportion to the degree of hearing loss and depending on the patient'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 patient with hearing loss for their concomitant somatic pathology. Medical treatment of cardiovascular diseases, nervous system disorders, endocrine pathology, and immune status abnormalities simultaneously serves as treatment and prevention of the progression of chronic sensorineural hearing loss in these patients.
In the presence of vestibular disorders and subjective tinnitus associated with chronic sensorineural hearing loss, pathogenetic and symptomatic treatment is administered.
In the presence of sensorineural hearing loss in patients with visual impairment and other disabling conditions, the indications for hearing aid fitting are expanded, and the requirements for assistive hearing devices increase.
The inability to achieve an effect using hearing aids and cochlear implants necessitates alternative communication methods—teaching the basics of fingerspelling and sign language, and sign language interpretation.
Criteria for Evaluating the Quality of Medical Care
№ | Criterion | Evaluation of compliance |
|---|---|---|
Performed: pure-tone audiometry, tympanometry (outpatient). | Yes/No | |
Initial general practitioner consultation (examination) (for H90.5, H91.2) performed (outpatient). | Yes/No | |
Initial endocrinologist consultation (examination) (for H90.3, H90.5, H91.2, H91.8) performed (outpatient). | Yes/No | |
Initial neurologist consultation (examination) (for H90.3, H90.4, H90.5, H91.2, H91.8) performed (outpatient). | Yes/No | |
Biochemical blood analysis for lipid metabolism disorders (cholesterol, LDL, HDL); complete blood count (CBC) with differential; general clinical biochemical blood analysis (glucose level, total and direct bilirubin, ALT, AST, creatinine, urea); coagulography (screening hemostasis test) (aPTT, INR, prothrombin time, fibrinogen) (outpatient or inpatient). | Yes/No | |
Pure-tone audiometry performed no later than 24 hours from the first presentation (for H90.5, H91.2) (outpatient). | Yes/No | |
Initiation of drug therapy no later than one month from diagnosis (for H90.5, H91.2). | Yes/No | |
Glucocorticoid therapy administered as part of conservative treatment (in the absence of medical contraindications) (for H90.5, H91.2) (inpatient). | Yes/No | |
Restoration of hearing thresholds to normal achieved (for H90.5, H91.2). | Yes/No | |
Restoration of hearing thresholds achieved, but not reaching normal levels (for H90.5, H91.2). | Yes/No | |
Stabilization of the condition achieved (no further progression of hearing loss). | Yes/No | |
Performed: pure-tone audiometry (according to the dispensary monitoring schedule) (for H90.3, H90.4) (outpatient). | Yes/No | |
Determination of indications for correction of impaired auditory function by traditional hearing aid fitting (H90.3, H91.8) performed (outpatient). | Yes/No | |
Determination of indications for correction of impaired auditory function by implantable hearing devices (including cochlear implantation) performed (outpatient). | Yes/No | |
Determination of indications for referral to medical-social expertise and drafting of an individual rehabilitation program performed (outpatient). | Yes/No | |
Evaluation of hearing aid fitting efficacy performed if the patient is a user of an assistive hearing device (outpatient). | Yes/No | |
Differential diagnosis procedures (vestibulometry, auditory brainstem response testing) performed to rule out retrocochlear pathology (outpatient). | Yes/No | |
Magnetic resonance imaging (MRI) and computed tomography (CT), including contrast-enhanced scans, of the internal auditory canals, cerebellopontine angles, and posterior cranial fossa (outpatient or inpatient). | Yes/No | |
Consultation by a neurosurgeon in case of suspected acoustic neuroma or other space-occupying lesion, and by a neurologist in the presence of neurological pathology (H90.4) performed (outpatient). | Yes/No | |
Indications for correction of impaired auditory function determined (outpatient). | Yes/No |
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Appendix A1. Working Group Composition
Tavartkiladze Georgiy Abelovich, MD, PhD, Professor, medical association member, no conflict of interest
Machalov Anton Sergeevich, PhD, medical association member, no conflict of interest.
Nikitin Mikhail Vladimirovich, MD, PhD, Chief freelance specialist in sanatorium-resort treatment of the Russian Ministry of Health, no conflict of interest.
Boboshko Mariya Yurevna, MD, PhD, medical association member, no conflict of interest
Sapozhnikov Yakov Mikhaylovich, MD, PhD, Professor, medical association member, no conflict of interest.
Karneeva Olga Vitalievna, MD, PhD, Professor, medical association member, no conflict of interest.
Kuznetsov Aleksandr Olegovich, MD, PhD, medical association member, no conflict of interest.
Abdulkerimov Khiyir Tagirovich, MD, PhD, Professor, medical association member, no conflict of interest.
Kovlen Denis Viktorovich, MD, PhD, Head of the Department of Physical and Rehabilitation Medicine, no conflict of interest.
Abuseva Gyulnara Ryakitovna, Lecturer at the Department of Physical and Rehabilitation Medicine, no conflict of interest.
Tsygankova Evgeniya Rostislavovna, PhD, Associate Professor, medical association member, no conflict of interest
Klimantsev Sergey Aleksandrovich, PhD, Associate Professor, medical association member, no conflict of interest
Developing institutions:
National Medical Research Center for Otorhinolaryngology of the Federal Medical-Biological Agency of Russia.
Russian National Research Centre for Audiology and Hearing Prosthetics of the Federal Medical-Biological Agency of Russia
Russian Medical Academy of Continuous Professional Education, Department of Audiology
Pirogov Russian National Research Medical University of the Russian Ministry of Health, Department of Otorhinolaryngology of the Faculty of Additional Professional Education
Ural Medical Academy, Department of Otorhinolaryngology
Hearing and Speech Laboratory of the Research Centre of the Pavlov First Saint Petersburg State Medical University
North-Western State Medical University named after I.I. Mechnikov
Kirov Military Medical Academy of the Russian Ministry of Defense, Department of Physical and Rehabilitation Medicine.
Sanatorium and Resort Complex "Vulan" – Scientific and Clinical Branch of the National Medical Research Center for Rehabilitation and Balneology of the Russian Ministry of Health
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 Order of the Ministry of Health No. 1183n dated December 20, 2012 "On Approval of the Nomenclature of Medical Worker Positions"):
1. Otorhinolaryngologists-audiologists;
2. Otorhinolaryngologists;
3. General practitioners, neurologists, general practice physicians (family physicians).
Table 1. Level of Evidence (LoE) grading scale for diagnostic methods (diagnostic interventions)
LoE | Definition |
|---|---|
1 | Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis |
2 | Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis |
3 | Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case report |
5 | Only justification of mechanism of action or expert opinion is available |
Table 2. Scale for assessing levels of reliable evidence (LRE) for prevention, treatment, and rehabilitation methods (preventive, therapeutic, rehabilitation interventions)
LoE | Definition |
|---|---|
1 | Systematic review of RCTs with meta-analysis |
2 | Individual RCTs and systematic reviews of studies of any design except RCTs, with meta-analysis |
3 | Non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case reports or case series, case-control studies |
5 | There is only a rationale for the mechanism of action of the intervention (preclinical studies) or expert opinion |
Table 3. Grade of Recommendation (GoR) scale for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitative interventions)
GoR | Definition |
|---|---|
A | Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent) |
B | Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent) |
C | Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent) |
Procedure for updating clinical guidelines.
The update mechanism for clinical guidelines provides for their systematic revision—at least once every three years, as well as upon the emergence of new evidence-based data regarding the diagnosis, treatment, prevention, and rehabilitation of specific conditions, 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
Procedure for the provision of medical care in the profile «otorhinolaryngology»: Order of the Ministry of Health of the Russian Federation No. 905n dated November 12, 2012, «On approval of the Procedure for the provision of medical care to the population in the profile „otorhinolaryngology“».
Procedure for the provision of medical care in the profile "audiology-otorhinolaryngology": Order of the Ministry of Health of the Russian Federation No. 178n dated April 9, 2015 "On Approval of the Procedure for the Provision of Medical Care to the Population in the Profile "Audiology-Otorhinolaryngology".
Letter of the Ministry of Health and Social Development of the Russian Federation No. 2383-RK dated April 1, 2008 "On Universal Audiological Screening of Infants in the First Year of Life".
Preoperative evaluation and surgical treatment of patients with grade IV sensorineural hearing loss and deafness: clinical guidelines. Moscow, 2015.
Rehabilitation of patients after cochlear implantation: clinical guidelines. Moscow - St. Petersburg, 2014.
Appendix B. Physician Action Algorithms
Adults
Determination of indications for hearing aid fitting (stable hearing thresholds exceeding the averaged speech range threshold by 31 dB HL) taking into account the patient's age, occupation, educational needs, and social activity.
Determination of indications for referral for medical and social expertise, taking into account hearing aid fitting capabilities and the level of speech development.
Evaluation of the effectiveness of hearing aid fitting using speech audiometry and questionnaires.
Determination of indications for implantable prosthetic forms, including cochlear implantation.
Appendix C. Patient Information
Treatment issues are of particular urgency and emergency in acute sensorineural hearing loss. The lack of effective treatment for chronic sensorineural hearing loss from an evidence-based medicine perspective requires the timely initiation of impaired auditory function correction.
In unilateral hearing loss, additional differential diagnostic procedures are required.
If sensorineural hearing loss is accompanied by vestibular disorders and/or subjective tinnitus, this requires additional diagnostic, therapeutic, and rehabilitation measures.
Based on indications, the patient is referred to a medical and social expert evaluation board to determine permanent disability. The disability group is assigned along with the drafting of an individual rehabilitation program (IRP) in accordance with the recommended type of correction (traditional hearing aids, implantable hearing aids, cochlear implantation). The patient is informed about the procedure for obtaining high-tech medical care (Otolaryngology 1).
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
Appendix G1. International Outcome Inventory for Hearing Aids
Russian title: International Outcome Inventory for Hearing Aids
Original title (if available): The International Outcome Inventory for Hearing Aids (IOI-HA)
Source (official developer website, validation publication): [75]
Type (underline): rating scale
Purpose: International assessment of hearing aid use
Content (template):
1. Think about how long you have used your hearing aid(s) over the past two weeks. How many hours a day do you typically use the aid(s)?
Do not use | Less than 1 hour a day | 1 to 4 hours a day | 4 to 8 hours a day | More than 8 hours a day□ |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
2. Think of a situation where you really wanted to hear better before you got your hearing aid(s). Over the past two weeks, how much did the aid(s) help in these situations?
Did not help at all | Helped slightly | Helped moderately | Helped quite a bit | Helped very much |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
3. Think again about a situation where you really wanted to hear better. When using your hearing aid(s), how much of a problem do you STILL have in this situation?
Very much a problem | Quite a bit of a problem | Moderate problem | Slight problem | No problem |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
4. Considering everything, do you feel your hearing aid(s) are worth the trouble?
Not at all worth it | Hardly worth it | Worth it, perhaps | Worth it | Definitely worth it |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
5. Over the past two weeks, with your hearing aid(s), how much has your hearing difficulty affected your ability to do things you wanted to do?
Affected very much | Affected quite a bit | Affected moderately | Affected slightly | Did not affect at all |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
6. Over the past two weeks, with your hearing aid(s), how much do you think your hearing difficulty has bothered other people?
Very severely troubled | Severely troubled | Bothered quite a bit | Bothered slightly | Did not bother at all |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
7. Considering everything, how much has your present hearing aid(s) changed your enjoyment of life?
Made it worse | Did not change it | Slightly improved it | Quite a bit improved it | Very much improved it |
|---|---|---|---|---|
□ | □ | □ | □ | □ |
Key (interpretation): If response options 1 and 2 are chosen across all 7 questions, adjustment of hearing aid settings is required, hearing testing should be repeated, and the patient should be referred for a course of auditory-verbal rehabilitation. If option 3 is chosen for all answers, intervention in hearing aid settings is required. If option 4 or 5 is chosen, no changes to hearing aid settings are required.