Medical knowledge | Otorhinolaryngology, ear, nose, throat » Hearing Disorders in Children and Adults

Hearing Disorders in Children and Adults [41] 1 Importance of hearing • Communication is based on speech and hearing • Continuous contact with the environment • Consequences of severe deafness in childhood  delayed development of speech, reading difficulties, intellectual and emotional disorders, behavioral disturbances, articulation problems • Severe deafness in adults Isolation, depression, secondary speech disorders [41] 2 Statistical data • WHO (2001): 50 million hard of hearing patients all over the world, 30% severely deaf, 20% children • 10% of the population is involved, 5-6% needs rehabilitation • Incidence of hearing disorders in infants in Hungary is 1% (=1000 newborn/year must be rehabilitated!) • Distribution of hearing disorders according to localization: -sensorineural: 60% -conductive: 30% -mixed: 10% [41] 3 Basics of audiology • The decibel (dB) is a relative value, which compares one sound pressure to another. dB=10 log I/I0

• 1 dB=1.26x • 3 dB=2x • 10 dB=10x • 20 dB=100x • 60 dB=1 000 000-fold increase of intensity, etc. • Hearing range 0-120 dB (20μPa-20Pa/1000 Hz) • Hearing loss: -normal 0-25 dB -slight deafness 25-40 dB -moderate deafness 41-60 dB -severe deafness 61-90 dB -profound deafness >90 dB -total deafness no hearing at all [41] 4 Normal pure tone threshold audiogram [41] 5 What do you have to think of if the patient complains about sudden deafness? • If there is no other symptom: wax (impacted cerumen) • If there is pain, too: otitis externa, furuncule of the external meatus, acute purulent otitis media (adults) • If pain is accompanied with fever: acute purulent otitis media (children) • If there is feeling of pressure and fullness in the ear accompanying a common cold: tubal occlusion • If there is also severe vertigo and discharge: labyrinthitis (complication of chronic otitis) • If there is also tinnitus and vertigo: acute hearing loss

(microcirculation disorder of the inner ear), acoustic neuroma • If there is also an attack of vertigo and tinnitus: Meniere`s disease • If there is also trauma in case history: perforation of the tympanic membrane, labyrinth commotion, pyramidal fracture, [41] 6 acute acoustic trauma What do you have to think of if the patient complains about progressive hearing loss? • Presbyacusis: symmetrical bilateral hearing loss in older patients, speech comprehension is diminished, recruitment • Noise induced hearing loss : there is also tinnitus, hearing loss is affected by exposition time/intensity/quality of noise • Otosclerosis: in young (female) patients, often bilateral, familial disposition, tinnitus • Hereditery deafness: family history • Acoustic neurome: tinnitus, vertigo, in some cases facial palsy • Chronic purulent otitis media: discharge, perforation of the tympanic membrane • Tympanosclerosis, adhesive process [41] 7 What is an emergency at a hearing

impaired patient? • Suspicion of acute hearing loss • Trauma (skull base fracture, perforation of tympanic membrane, acoustic trauma) • Pain (otalgia)+discharge+vertigo+facial palsy • Pain+discharge+vertigo • Pain+discharge+fever • Pain+discharge • Pain [41] 8 Diagnosis of hearing loss • Case history (family history, special ENT history, medications, trauma, noise, accompanying symptoms, internal diseases, etc.) • ENT physical examination • Audiological examination (pure tone threshold, speech audiometry, tympanometry, stapedial reflex, objective audiometry, otoacoustic emission) • Radiological assessment (Schüller`s view, CT,MR) • Otoneurology • Neurology • Internal diseases • Laboratory examinations [41] 9 Characteristical hearing losses • High frequency hearing loss: acoustic trauma, presbyacusis • Low frequency hearing loss: M. Meniere, otitis media, otosclerosis • Speech discrimination loss: lesion of the inner ear or n.VIII •

Fluctuating hearing loss: M. Meniere, acute hearing loss • Better hearing in noise: conductive hearing loss • Unilateral hearing loss: M. Meniere, acoustic neurinome • Bilateral hearing loss: presbyacusis, noise induced hearing loss, ototoxic lesions [41] 10 What do we have to ask from the patient? • • • • • • Since when does the patient have hearing loss? Is it unilateral or bilateral ? Is it permanent or intermittent? How severe is the hearing loss? Is there a discharge, tinnitus, vertigo, pain? Did the patient have ear surgery or any otological event previously? [41] 11 Etiology and incidence of hearing losses 12% 11% [41] 23% 22% 12% presbyacusis ismeretlen idegi chronicus ot. fertőzés veleszületett M.Meniere egyéb 11% zajártalom tympanosclerosis trauma otoscler. toxikus acut 12 [41] 13 Conductive hearing loss • • • • Etiology: diseases of external and middle ear Usually surgically treated Speech is soft and monotonous Good

speech comprehension with appropriate amplification • No significant articulation disorder [41] 14 Conductive hearing loss [41] 15 Conductive hearing loss 1. • Wax (use of cotton Q-tips is forbidden!) • Foreign body (it mustn`t be removed with a simple forceps!) • Severe otitis externa (importance of local treatment, exclusion of diabetes, must avoid irritants and Q-tips!) • Congenital or acquired meatal atresia, ossicular abnormalities) • Osteoma, exostosis • Tumors of the external and middle ear [41] 16 Foreign body in the external meatus (bead) [41] 17 Cerumen [41] 18 Multiple exostoses in the ear canal [41] 19 Conductive hearing loss 2. • Traumatic perforation of the tympanic membrane (must avoid water! Must be examined by a specialist, often closes spontaneously) • Tubal catarrh, serosus otitis media (usually during/after a common cold, caused by adenoid vegetation in case of children, in adults tumor of the epipharynx must be

excluded!) • Acut purulent otitis media (decongestant nasal drops, local heat, ATB, myringotomy in selected cases, if hearing loss persists for more than 4 weeks=surgery) • Chronic purulent otitis media (perforation, discharge, hearing loss=surgery!) [41] 20 Serous otitis media [41] 21 Acute purulent otitis media [41] 22 Chronic mesotympanic otitis media [41] 23 Conductive hearing loss 3. • Otosclerosis (localized disease of bone metabolism in the labyrinth bony capsulecauses ankylosis of the stapes in the oval window, in young patients, usually bilateral, often in family history, stapedectomia/stapedotomia) • Tympanosclerosis, adhesive process (consequence of insufficiently healed inflammations, importance of prevention!) • Disruption of the ossicular chain (after previous operations or myringotomy) • Longitudinal pyramidal fracture (importance of previous otological case history and tuning-fork findings ) [41] 24 Tympano sclerosis [41] 25

Sensorineural hearing loss • Etiology: diseases of inner ear and upper auditory pathways • Usually treated with conservative methods (hearing aids, infusions, vitamins), but might be operated in selected cases • Speech is much too loud • Speech comprehension problems • In severe, untreated cases articulation problems [41] 26 Sensorineural hearing loss [41] 27 Sensorineural hearing loss 1. • Congenital inner ear malformations (Michel, Mondini) • Hereditary hearing loss or deafness -sporadic -progressive familial -combined with other inherited symptoms (Usher/retinitis pigmentosa, Alport/chronic nephritis, Pendred/thyroid gland disorders, etc.) • Prenatal acquired (rubella, syphilis, toxoplasma, herpes zoster, CMV, toxic damages, fetal hypoxia, diabetes, irradiation) • Perinatal (fetal asphyxia, erythroblastosis foetalis) [41] 28 Sensorineural hearing loss 2. Postnatally acquired hearing losses • Trauma (perilymph-fistule, labyrinth commotion,

pyramidal transverse fractures) • Noise-induced (acoustic-trauma, noise-induced hearing loss) • Presbyacusis • Toxic lesions (exogen toxins:aminoglycosids!!!, industrial toxins, diuretics, cytostatics, nicotin, alcohol, salycilates, quinine, dihydrostreptomycin, endogen toxins: toxic metabolits of renal disease and diabetes mellitus) • Microcirculation disorders (hypertonic disease, arteriosclerosis, diabetes, lipid metabolism disorders, nicotin, vertebrobasilar insufficiency) • Infections (meningitis, mumps, herpes zoster, measles, scarlatina, lues, tbc, Lyme-disease, influenza) • M. Meniere • Tumors (acoustic neuroma), metastasis, congenital cholesteatoma • Sclerosis multiplex [41] 29 Herpes zoster oticus [41] 30 Noise-induced hearing loss [41] 31 Presbyacusis [41] 32 Recruitment phenomenon [41] 33 Speech audiogram [41] 34 Sensorineural hearing loss 3. Therapy • Importance of prevention (noise, ototoxic antibiotics) • Acute

hearing loss: vasodilatator infusions, vitamins, corticosteroids, treatment of internal diseases • Chronic hearing loss: hearing aid, in case of symmetrical hearing loss bilaterally • In selected cases of profound bilateral hearing loss of cochlear origin: cochlear implantation [41] 35 Mixed hearing loss [41] 36 Acute hearing loss • It is a medical emergency! • Symptoms: feeling of pressure in the ear tinnitus severe hearing loss or complete deafness • Usually caused by a disorder of microcirculation of the inner ear (occasionally may be an autoimmun disease) • Diagnosis: generally unilateral sensorineural hearing loss with recruitment • Differencial diagnosis: acoustic neuroma, acute tubal catarrh, impacted cerumen, viral infections (mumps, herpes zoster, influenza) • Immediate vasodilatation or low-molecular weight infusions, if an autoimmune disease is suspected, prolonged corticosteroids are given [41] 37 Hearing loss in childhood When to suspect it?

• If a newborn consistently doesn`t react to noise • If the tone of crying is unusual • Delayed development of speech • Slurred articulation • Acoustic inattention • Unusual behaviour (introversion or aggressivity) [41] 38 Risk factors of hearing loss in childhood • • • • • Intrauterin, peri- és postnatal infections Neonatal hyperbilirubinaemia Craniofacial disorders Hearing loss in family history Symptoms of well known syndromes combined with hearing loss • NICU treatment lasting more than 48 hours • Syndromes with progressive hearing losses • Neurodegeneratíve illnesses • [41]Parental suspicions 39 Diagnosis and treatment of hearing impaired children • In case of suspicious symptoms and risk factors immediate assessment of hearing (and treatment) is needed! • Infancy: objective measurements (otoacoustic emission, stapedial reflex, auditory brainstem audiometry) • Early childhood: playaudiometry and objective measurements •

Schoolchildren: pure tone audiometry may be performed • Immediate therapy according to cause and degree of hearing loss • Aim: prevention of auditory deprivation, acquiring speech, hearing training, attending normal schools • In case of profound bilateral inner ear deafness, which can`t be corrected with hearing aids: early cochlear implantation (in 2-3 years [41] of age) 40 Prevention of hearing loss • Protection of hearing against noise, systematical screenings • Avoiding ototoxic antibiotics (aminoglycosids), in case of need close monitoring (renal function!) • In case of acute hearing loss immediate hospitalisation • Introduction of systematical screening routinely from the time of birth in all over the country • Close observation of children with risk factors • Rehabilitation of patients with hearing aids • Education of people about hearing [41] 41