Etiology, clinical forms and methods of the sensorineural hearing loss treatmеnt (review of the literature)

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Etiology, clinical forms and methods of the sensorineural hearing loss treatment (review of the literature)


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2. Syggelos S.A., Giannopoulou E., Gouvousis P.A., Andonopoulos A.P., Aletras A.J. Panagiotopoulos E. In vitro effects of non-steroidal anti-inflammatory drugs on cytokine, prostanoid and matrix metalloproteinase production by interface membranes from loose hip or knee endoprostheses. //Osteoarthritis Cartilage. 2007 May-15 (5): 531−42. Epub 2006 Dec 22.

3. St'-ovickova J, Hulejova H, Pesakova V, Spacek P, Klezl Z, Trc T, Adam M. Relation between cytokines, adhesive immunoglobulins and matrix metalloproteinases in osteoarthritic joints//J. Rheumatol. 2009 Aug- 36 (8): 1618−25. Epub 2009 Jun 16.

4. Klett R., Kordelle J. Stahl U., Khalisi A., Puille M., Steiner D., Bauer R. Immunoscintigraphy of septic loosening of knee endoprosthesis: a retrospective evaluation of the antigranulocyte antibody BW 250/183. //Eur J Nucl Med Mol Imaging. 2003 Nov-30 (11): 1463−6. Epub 2003 Jul 22.

5. Fuchs D., Zinman C., Lewinson D. Characterization of the biological process of aseptic loosening of joint implants in orthope-dics//Harefuah. 1998 Mar 15−134 (6): 432−5, 504, 503.

6. Toom A., Arend A., Gunnarsson D., Ulfsparre R., Suutre S., Haviko T., Selstam G. Bone formation zones in heterotopic ossifications: histologic findings and increased expression of bone morphogenetic protein 2 and transforming growth factors beta2 and beta3. //Calcif Tissue Int. 2007 Apr-80 (4): 259−67. Epub 2007 Apr 1.

7. Reznik L. B., Turushev M. A. About possible optimization of stress bone remodeling in endoprosthesis of the knee joint//Vestnik travmatologii I jhnjpedii im. N. N. Priorova, 2009, № 4. — P. 19−23.

8. Volokitina E. A., Chepeleva M. V. Laser flow cytometry in the laboratory immunologic monitoring of the endoprosthesis of the knee and hip joints//Genius Ortopedii № 3, 2011. — P. 131−135.

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Amonov Aminjon Shavkatovich, attending medical doctor «Department of congenital and acquired diseases of ENT organs» Republican specialized scientific-practical medical center of Pediatrics, Uzbekistan

E-mail: amonov. aminjon@mail. ru

Etiology, clinical forms and methods of the sensorineural hearing loss treatment (review of the literature)

Abstract: Sensorineural hearing loss (SHL, synonym — Neurosensory Hypoacusis) is hearing loss caused by damage of the inner ear structures, vestibulocochlear nerve (VIII), or central parts of the auditory analyzer (in the stem and the auditory cortex) [1]. It is one of the most relevant and complex medical and social problems of otorhinolaryngology. Conservative SHL treatment still remains ineffective and the searches for new efficient methods are demanded.

Keywords: hearing loss, causes, sensorineural hearing loss, classification, treatment.

According to the World Health Organization (WHO) globally hearing loss is one of the six leading factors that can impair quality of life [22]. According to WHO (2012) 360 million people (5. 3% ofthe population) suffers from the auditory function violation, 328 million of this people — adults, and 32 million — children. In 80% ofpatients with acoustic disturbance the hearing loss is caused by sound-perceiving apparatus damage [3, 4, 16]. WHO stated that by 2020 the number ofpeople with acoustic disturbances increased not less than 30%.

The term hearing loss means hearing reduction which can be mild (the auditory threshold of sounds with frequency 5 004 000 Hz is on the level of 30−40 dB), moderate (50−60 dB) and severe (more than 60−70 dB).

In case of mild hearing loss the whisper speech comprehension is possible from the distance of 4−6 m, in case of moderate hearing loss — from 1 to 4 m, in severe — 0. 25 to 1 m. The greater hearing loss is defined as deafness [1]. Sometimes, when the auditory threshold of the oral speech increases up to 70−90 dB, we can think about profound hearing loss, and over 90 dB — about the deafness.

The most common is SHL caused by the auditory receptor cell lesion. Rarely sensorineural hearing loss occurs associated with vestibulocochlear nerve (VIII cranial) or auditory brain center damage. And in very rare cases only central parts ofacoustic analyzer (usually in pathological lesions ofthe cerebral cortex temporal lobe, where the cortical center ofhearing is localized and associated) are damaged [10].

The SHL can be congenital (CSHL) or acquired (ASHL) and hereditary.

The cause of congenital SHL can be the following:

• Insufficient development (aplasia) of the cochlea, including autosomal-recessive Shaibe aplasia, autosomal-dominant Michael and Mondini aplasia.

• Syndromal (combined with the other pathological symptoms) sensorineural hearing loss caused by chromosome defects (rare).

• Congenital cholesteatoma. This is hyperplasia of the middle ear squamous epithelium, manifested as invasive tumor, which, during its growth, destroys the middle and inner ear structures with cochlea receptor apparatus damage.


Section 6. Medical science

• Fetal alcohol syndrome contributes development of hearing loss in 64% of children which have been borne from mothers suffering from alcoholism due to ototoxic effect on the developing fetus and insufficient absorption of nutrients due to excessive alcohol consumption [5].

• Preterm births lead to sensorineural deafness in approximately 5% of cases [5].

• Chlamydia may cause hearing loss in newborns, infected from mother during childbirth [5].

• Syphilis is typically passed from a pregnant woman to the unborn child, and about one-third of infected children subsequently become deaf[5].

• Congenital rubella syndrome is manifested in the form of so-called Greg triad (sensorineural deafness, congenital heart disease, severe eye diseases) in case of intrauterine infection.

The acquired SHL develops in the process of human development in the postnatal period. Factors contributing to hearing loss at this age can be:

• Ototoxic medications. The most dangerous are aminoglycoside antibiotics (the main drug — gentamicin). They selectively accumulate in the endo — and perilymph, and in combination with diuretics can fatally affect sensorineural structures of the inner ear [1]. This group of drugs includes tobramycin, streptomycin, monomitsin, kanamycin, and amikacin. There are also drugs that cause reversible hearing loss. It is a series of diuretics, cytostatics, and macrolide antibiotics.

• Viral and bacterial infection is one of the dominant causes of acquired SHL. Such as measles, rubella, influenza, and herpes often lead to a bilateral lesion of the inner ear [1].

Epidemic parotitis (mumps) may result in profound sensorineural hearing loss and deafness with increasing of the individual auditory threshold up to 90 dB or more, unilateral (one ear) or bilateral (both ears).

In patients with AIDS or HIV hearing loss are often can be found, because HIV and associated with it opportunistic infections may directly affect the cochlea and central parts of the auditory system [5]. SHL can develop in patients with acute and chronic viral hepatitis. Clinical observation have shown significant hearing loss or deafness development in case of cirrhosis, uremia, diabetes mellitus, as a result of the increased level of oxidized metabolism products and content of the toxic substances in the blood. The hearing impairment can be caused by the inner ear inflammation (purulent labyrinthitis) and meninges inflammation (meningitis).

• The adenoid vegetations, which have not disappeared in adolescence, may increase in the future, resulting in reduced passability of the Eustachian tube, conductive hearing loss and development of the infections of nasal cavity, which can pass into the inner ear.

• One of possible allergic rhinitis complications — chronic serous otitis media — is the most common cause of hearing loss in children in the USA [2]. Researchers have registered acute hearing loss of the allergic origin in case of local use of sea buckthorn oil for the treatment of inflammation of the nasal cavity [7].

• Autoimmune diseases, as it recently has been revealed, can cause cochlea damage. Although the cases where the cochlea structures are the only specific target of autoimmune processes most probably are very rare- usually pathological changes also affect other organs. Thus, autoimmune diseases that cause multiple diseases and can cause the hearing loss, including Wegener'-s granulomatosis.

• Tumors of the cerebellopontine triangle. The cerebellopontine triangle (angle) — area of the brain between the pons Varolii and the cerebellum. This area includes places of the facial nerve

(VII) and vestibulocochlear nerve (VIII). The objective signs and symptoms of both nerves compression often can be observed in patients with such tumors.

• The eighth nerve tumor — vestibular schwannoma- a benign tumor, emerging due to neurilemma cells fission.

• Meningioma is a benign tumor of the meninges or arachnoid membranes of the brain.

• Otosclerosis is characterized by abnormal growth of bone around the stirrup-shaped bone — one of the middle ear auditory ossicles, which leads to the ossicles immobility and conductive hearing loss. Subsequently otosclerotic process may also occur in different parts of the inner ear, causing sensorineural hearing loss [1].

• Meniere'-s disease causes the SHL in the low frequency range (125−1000 Hz). The attacks of sudden dizziness, lasting from several minutes to several hours, preceded by tinnitus, feeling of their blockage and unstable hearing loss.

• The presbycusis. This is the hearing impairment due to loss of reception of high tones (high frequency range 4000−8000 Hz), observed mainly in the elderly people. It is considered by several researchers as a degenerative process, although currently the conditionality of this disease by the age-related changes are stiff not proven.

• Acoustic trauma — prolonged exposure to loud noises (& gt-90 dB) causes hearing loss in the high frequency range 4,000 Hz, whereas a normal hearing frequency range varies from 20 to 20 000 Hz. Those peoples which leave near the airports or expressways exposed to noise (typically 65 to 75 dB according to the A scale). If windows in the room are constantly opened, or person spends a lot of time outdoors, such noise exposure can lead to a hearing loss.

• Mechanical traumas — fractures and fissures of the temporal bone affecting the cochlea and middle ear, or incised wounds with VIII cranial (auditory) nerve damage can lead to the sensorineural hearing loss development.

• In case of trauma can appears not only the ear structures damage, but also damage of the brain auditory centers that process sound information.

Sometimes the sudden SHL can appear. This is a hearing loss in a very short time — up to 12 hours (for longer periods — up to one day — the pathology is most often defined as acute hearing loss) [1]. Causes of this condition may be as follows:

• Idiopathic sudden SHL when it is not possible to identify certain factors for the hearing loss development [20]-

• As a result of impaired inner ear blood supply (vascular ischemia) [12- 13]-

• Due to the perilymphatic fistula formation — usually arises due to round or oval window perforation and is accompanied by the perilymph outflow- dizziness and loss of balance are typical- there are increased intracranial pressure or traumas are in the anamnesis.

• Sudden sensorineural hearing loss can be triggered by allergic reactions caused by immunoglobulin E (IgE) to food, pollen, fungal spores and other allergens.

• The so-called stress hearing loss, that is one of the sudden hearing loss forms, is the basis of which lies sudden vasoconstriction.

Hearing loss can be hereditary [6- 8- 14- 15- 18- 21]. There are dominant and recessive genes contributing the hearing loss development — from mild to profoundly severe. If at least one parent has a dominant autosomal gene causing deafness, the hearing loss will obligatory manifest in progeny. Hearing impairment caused by a recessive gene does not always manifest, but only in those progenies which have received recessive genes both from mother and from father.


Etiology, clinical forms and methods of the sensorineural hearing loss treatment (review of the literature)

Hearing loss in case of either dominant or recessive heredity types may manifest in the form of various syndromes, but not always. Thus gene mapping, carried out in recent years have revealed dozens of cases of nonsyndromic (not coupled with other anomalies) autosomal dominant (DFNA#) and autosomal recessive (DFNB#) deafness.

• Among hereditary hearing impairment the autosomal recessive nonsyndromic deafness (DFNB1) which is associated with gene GJB2, encoding the protein connexin 26, mutation is the most common in developed countries.

• The most common syndromic autosomal-dominant hearing losses are Stickler syndrome and Waardenburg (Wardenburg) syndrome. Rare diseases with an autosomal dominant inheritance, leading to sensorineural hearing loss, include autoinflammatory Muckle — Wales syndrome.

• The most common syndromic hearing loss, associated with autosomal-recessive inheritance, includes Pendred'-s syndrome (congenital sensorineural deafness and goiter) [11], a wide aque-ductus vestibuli syndrome and Usher'-s syndrome (congenital sensorineural deafness and retinitis) [11]. The Jervell — Lange-Nielsen (congenital sensorineural hearing loss and functional abnormalities of the heart) have the same mode of inheritance. It occurs in populations with a frequency of1: 100 000−1: 200 000, most of the patients do not survive to the age of 15 years [11].

• The mitochondrial gene MT-TL1 mutations can cause diabetes mellitus, accompanied by deafness.

In recent years, approaches to etiopathogenetic classification of the SHL, that are used in audiology, especially in children'-s audiology, became more differentiated. Thus, the factors responsible for the development of hearing impairment in children, i. e. the causes of hearing loss, depending on the time of exposure are divided into the antenatal, intranatal and postnatal [9- 17- 19- 20].

• Antenatal and intranatal causative factors lead to the congenital ear lesions formation, and postnatal — to early hearing loss. All acoustic disturbances in children, which occur in perinatal period, are considered congenital. Genetic factors of acoustic disturbances have been noted, when hearing loss or deafness occur in someone from children'-s close relatives- up to 50% of deaf children have pathology of hereditary nature.

• Hereditary hearing loss include a large variety of genetic syndromes. Identification of genes responsible for hearing loss is possible, but genetically accurate diagnosis can be rarely defined.

• In case of genetic pathology, the hearing loss often manifests in the first or second decade of life and is exacerbated with the age, and at the child birth differential diagnosis of syndromic and nonsyndromic hearing losses is complicated.

Among antenatal adverse factors acting on the fetus during the prenatal period, can be noted:

• Pathological pregnancy course (gestational toxicosis of I and II trimester, nephropathy, threatened miscarriage, anemia, RBC sensitization, etc.) —

• Mother'-s viral and bacterial infectious diseases during pregnancy, which primarily include cytomegalovirus and herpetic infections, influenza, rubella, and toxoplasmosis-

• Mother'-s somatic diseases (diabetes mellitus, cholesteremia, cardiovascular disease, kidney disease, etc.) —

• Treatment by ototoxic drugs during the pregnancy — antibiotics (aminoglycosides), diuretics (furosemide, ethacrynic acid), salicylates-

• Alcohol consumption, drug consumption, smoking, effect of some agricultural and industrial chemicals and radiation during pregnancy, etc-

Intranatal causes include:

• Effect of the adverse factors leading to neonatal asphyxia, and intracranial birth injury-

• Precipitated labor, prolonged labor or premature birth-

• Breech presentation, pelvic presentation and face presentation-

• Maternity aid (forceps operation, vacuum extraction, caesarean section) —

• Bleeding during labor, abruption of placenta, danger of uterine rupture, etc.

Postnatal causes of hearing loss development in young child cause an adverse effect on the child'-s organism after the childbirth. They include hyperbilirubinemia, infection, sepsis, meningitis, meningoencephalitis, inflammatory diseases of middle and inner ear, exogenous ototoxin, prematurity, etc.

Summarizing the discussion of the SHL causes and clinical forms it is necessary to pay attention to the fact that one factor effect is usually not enough to damage the analyzer. Usually in case of pronounced, significant hearing loss, several factors must affect the organism for a long time. The hearing loss reversibility and success of treatment will also depend on these facts.

Treatment of sensorineural hearing loss:

• Previously the sensorineural hearing loss has been corrected only by the help of a hearing aid, amplifying sounds in the frequency range covered by the hearing loss. Now for these purposes more often cochlear implants are used, which directly stimulate the auditory nerve and in more than 90% of cases give positive results in terms of oral-aural rehabilitation of such patients.

• The earlier the cochlear implantation will be carried out, the sooner socially adequate hearing and speech will be formed.

• If more preferable cochlear implantation carrying out is impossible the brainstem implantation can be used, but the technology of such operations are still underdevelopment, and this kind of operations are performed infrequently.

• According to several audiologists and ENT-specialists severe sensorineural hearing loss caused by exposure to noise over 140 dB can often be almost completely cured, if promptly (before day expiry) perform a course of treatment with steroid drugs. However, the effectiveness of this new method requires further validation.

• Experiments with the stem cells taken from nasal mucosa of an adult transplantation into the inner ear cochlea structures of white mice, which has been carried out by the scientists from the University of New South Wales and Institute for medical research of Garvan (Sydney, Australia) have shown that at early stages of the sensorineural hearing loss development such surgical procedure conduce the auditory function preservation.


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3. Voronkin V. F., Lazarev L. A. Dynamics of immunological parameters and lymphocyte subpopulations during treatment of patients with acute sensorineural hearing loss: Mat. X VI Congress of otolaryngologists of Russian Federation «Otorhinolaryngology on the boundary of millennia& quot- SPb: «RIA-AMI& quot-, 2001. — P. 184−187.


Section 6. Medical science

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11. Stratieva O. V. Clinical anatomy of the ear: Training manual. St. Petersburg: 2004. — P. 271.

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13. Shidlovskaya T. V., Shidlovskaya T. A., Gerasimenko S. I. Qualitative indicators of encephalography in patients with initial sensorineural hearing loss on the background of various vascular factors. Otorhinolaryngology Eastern Europe, 2012, No. 3. — P. 13−21.

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19. Paludetti G., Gonti G., Di Nardo W., De Corso E., Piccioti P. M. Fetoni A. R. Infant hearing loss: from diagnosis to therapy Official report of XXI Conference of Italian Society of Pediatric Othorhinolaryngology. Acta Othorhinolaryngol Ital v. 32 (6) — 2012 Dec.

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22. Zahnert T. The differential diagnosis of hearing loss. Dtsch Arztebl int., 2011, vol. 108, N 25. P. 433−444.

Amonov Aminjon Shavkatovich, attending medical doctor «Department of congenital and acquired diseases of ENT organs» Republican specialized scientific-practical medical center of Pediatrics, the Republic of Uzbekistan

Vladislav Evgenevich Kuzovkov, Professor, head of the Department of diagnosis and rehabilitation of hearing impairments, Saint-Petersburg research Institute of ear, throat, nose and speech, the Russian Federation

E-mail: amonov. aminjon@mail. ru

Cochlear implantation in children with the inner ear congenital dysgenesia — mondini anomaly

Abstract: Cochlear implantation is a complex surgical intervention in children with inner ear anomalies. In patients with congenital sensorineural hearing loss the Mondini anomaly is most often diagnosed. During surgery it was found that using of the classical access and the introduction of the active electrode through the fenestra of the cochlea is possible in most patients with Mondini anomaly.

Keywords: inner ear anomaly, Mondini anomaly, cochlear implantation, children.


To evaluate the possibility and probable advantages and disadvantages of introducing an active electrode of cochlear implant through the fenestra of the cochlea in case of Mondini anomaly.

Materials and methods

The present study included 8 patients with inner ear malformations (Mondini anomaly) at the age of one year up to 5 years. All children underwent surgery with the diagnosis of severe congenital bilateral sensorineural hearing loss or deafness (in the RSRPMCP in 2014−2015 years).


The results of the study have shown that the classical access to cochlear implantation performance is possible in most patients with Mondini anomaly (75%) and the introduction of the active

electrode in this category of patients through the fenestra of the cochlea is almost always possible (87,5%).

Cochlear implantation (CI) is the only effective treatment for patients with severe sensorineural hearing loss and deafness [6- 7]. In most cases, the surgery is carried out using the standard method with the introduction of the active electrode through the fenestra of the vestibule or through cochleostomy. Often in case of SHL congenital anomalies of inner ear can be found. Patients with the inner ear malformations are the most difficult category of patients, both from the point ofview of the surgical intervention features, and from the point ofview of oral-aural rehabilitation [1- 2- 4]. The difficulty of the surgical intervention tactics choice in these patients is caused by inner ear anatomical structures large variability and therefore, the complexity in approaches to cochlear implantation standardiz-


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