Fluctuant Hearing Loss
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FLUCTUANT HEARING LOSS S. K. Jaiswal Assistant Professor Department of Ear, Nose and Throat S. K. Medical College Muzaffarpur, Bihar- 842003, India 122 cases of fluctuant hearing loss were studied. Serous otitis media was commonest followed by Meniere's disease, Acoustic neuroma, fistula of round and oval window, oto-mandibular syndrome, Congenital cholesteatoma and maxillary sinusitis. Cases of Arachnoid cyst, Multiple sclerosis, abnormalities of base of skull and craniocervical region and cervical rib causing fluctuant hearing loss could not be detected. Aetio pathophysiology of all discussed. INTRODUCTION (iv) Rare-cervical rib-irritating cervical sympathetic chain. Fluctuant hearing loss can be defined as the (v) Septic foci-most common maxillary sinusitis. hearing loss which has varying threshold at time intervals (at different time) in the same ear and is (3) Fluctuant neural (retro-cochlear) hearing loss reversible. It is a common clinical presentation Following conditions are neural fluctuant by patient in our day-to-day clinical practice. This hearing loss: common clinical complaint often accompanied (i) Acoustic neuroma. by sensation of fullness in the ear, roaring tinnitus (ii) Congenital cholesteatoma. and vertigo. The hearing threshold, with fluctuant (iii) Arachnoidal cysts. hearing, varies between two extremes, the normal (iv) Multiple sclerosis. or reduced stable hearing component, and the (v) Abnormalities of base of skull and cranio- superimposed fluctuant reversible component. cervical region. Three type of fluctuant hearing loss are clinically A precise description about aetiopathogenesis present. and pathophysiology in different otological (1) Fluctuant conductive hearing loss. diseases responsible for fluctuant hearing loss is (2) Fluctuant sensory (cochlear) hearing loss. being discussed here separately. (3) F l uctuant neural (Retro-cochlear) hearing loss. SEROUS OTITIS MEDIA 1. Yluctuant conductive hearing loss This most frequently encountered cause of Following conditions are the conductive fluctuant fluctuant conductive hearing loss of middle ear hearing loss: type is diJe to fluid in the middle ear. There is (i) Serous otitis media. temporary threshold shift which is smaller, as (ii) Oto mandibular syndrome. compared to normal, explains disturbance free 2. Fluctuant sensory (cochlear) hearing loss inner ear. Following condit'ions are the sensory fluctuant Arnold and Ganzer (1980) and Munker (I 980) hearing loss: advocates that there is almost always a sensory- neural hearing loss component in higher frequency (i) Round window fistula. range as well. This can be explained by two (ii) Oval window fistula. possibilities viz.: (iii) Meniere's disease. IJO & HNS Special Issue, 1997 67 Fluctuant Hearing Loss--S. K. Jaiswal (a) Diffusion of oxygen through the round as in round window fistula. window membrane into the inner ear might be MENIERE'S DISEASE affected to less or more extent. The most important disease of inner ear causing (b) Due to the mass loading effect of the fluid fluctuant hearing loss, vertigo, tinnitus and on the round window. ocassional vomiting is Meniere's disease. Hydrops This sensory-neural component (decreased of endolymph with poor reabsorption give rise to bone conduction threshold) immediately improves fluctuant sensory neural hearing loss, result of mass to a normal threshold after removal of fluid (serous loading effect of endolymph. or mucous from middle ear, is only a pseudo Nutritional, biochemical or mechanical change perceptive deafness (Huizing-1964). in the inner ear are thought to be responsible for OTO MANDIBULAR SYNDROME this sudden alteration in sensory function. Episodic leakage of endolymph into perilymphatic system This is a rare syndrome of pain in and around the cause symptom of disease Lawrence and McCabe ear, fullness in the ear, a fluctuant hearing loss, (1959), Schuknecht et al (1962). The potassium tinnitus and sensation of unsteadiness. This is which is rich in endolymph have toxic effect on caused by dysfunction of masticatory function for the afferent neurons of the eight cranial nerve, on longer period, produces spasticity of masticatory the other hand there is accumulation of sodium muscle and of tensor-tympani and tensor palati and other electrolytes in the endolymph. This may muscle as well, this causes change in impedance occur only after rupture of endolymphatic and dysfunction of eustachian tube function. This membrane, a questionable hypothesis. "tonic tensor tympanic-phenomenon of klockhoff and westerberg" (1973) is oto-mandibular Electron microscope showed that sealing syndrome or tensor tympani syndrome. Most of element between endolymph-perilymph barrier the patients suffers from psychological problems. are zonulae occludentes, the intercellular junctions, which surrounds the apical part of cell ROUND WINDOW FISTULA in a belt or band like manner. They influlence Sudden sensory neural fluctuant hearing loss, ion exchange along epithelial barrier. vertigo and roaring tinnitus are the features of Endolymphatic hydrops and its increased osmotic Labyrinthineg fistula. Mostly barotrauma or pressure destroy some of intra membranous fibrils physical strain diving etc., giving rise to sudden ofzonulae-occludentes. This leads to leakage of rise of CSF pressure causes rupture of round/oval endolymph into the perilymph (]ahnke-1977). window membrane. If medical treatment does not Endocrine disorder and disturbance in relieves symptoms within 10 days a microcirculation may lead to "diffuse membrane Tympanoscopy and surgical repair should be done leakage". to save patient from complete hearing loss. Management of Meniere's patient should be OVAL WINDOW FISTULA done after considering anatomy and pathophysiology. We can not alter the Having the same symptoms as round window pneumatization of the temporal bone, position of fistula aetiology of oval window fistula is different. sac and or perisccular fibrosis. So we have to Most common cause is post stapedectomy oval eliminate trigger factor during early stage of window fistula as compared to pressure change disease. A careful history should be taken for in middle and inner ear. stress, cigarette smoking, high salt intake, allergy In this, vestibular symptoms are more and psychological problem. The patient responds pronounced than hearing loss. Hearing loss is of to conservative medical treatment. On failure of both types, a fluctuant senory-neural and a stable conservative treatment to control vertiginous conductive hearing loss. Quality of tinnitus is same attacks, surgery is indicated. IJO & HNS Special Issue, 1997 68 Fluctuant Hearing Loss--S. K. Jaiswal ACOUSTIC NEUROMA brain stem compression and involvement of lower In small acoustic neuroma, sometimes, patient four cranial nerves. presents with fluctuant neural hearing loss. These Otological symptoms appear much before the patients present with slow progressive deafness. appearance of symptom and sign of brain-stem Some patients present with sudden deafness, compression and other cranial nerve involvement. which fully or partially iecovers with conservative Hence role of otologist is more important here treatment, shows fluctuant nature. This fiuctuant than neurologists and neuro-surgeons, as to neural hearing loss may be due to: diagnose it earliest. A plain lateral X-Ray or 1. Tumour affecting internal auditory artery tomograph can easily show cranio-cervical resulting interfe:ence with cochlear blood supply; dysplasia. Relationship of dens axis to Mc Gregor's or or Chamberlains line being most important. CT scan will also demonstrate the relationship in 2. Direct pressure on the cochlear nerve; or between dens or odontoid process and the 3. Secondary to marked increase in perilymph medulla oblogata. protein. Incidence of vertigo, fluctuant hearing loss and CONGENITAL CHOLESTEA TOMA tinnitus are the commonest findings in these cases Congenital cholesteatoma causes neural deafness of basilar impression. These symptoms differ from in some, not in all cases. It has been observed the Meniere's disease as these are related to head that in some cases full recovery of hearing loss movements and body flexion. has occurred after cholesteatoma was removed. Otological symptomalogy can be explained. This observation suggests about the enormous In platy basia disturbed circulation of CSF may potential of recovery of eighth nerve. be responsible for the symptoms. The Protruding odontoid process or dens axis may interfere with ARACHNOIDAL CYSTS inner ear function by irritating the perivascular Arachnoidal cysts are another cause of fluctuant autonomic nerves of the vertebral and basilar neural deafness. Surgery is required to remove arteries. The high dens and the reactive fi.brosis arachnoidal cyst. Recovery of hearing loss depends of the dura in adulthood lead to progressive upon damage caused by cyst. decrease in the calibre of the dura atthe foramen MULTIPLE SCLEROSIS magnum may cause otological symptoms. Multiple sclerosis may also cause fluctuant neural MATERIAL AND METHOD hearing loss. The demyelinating process is In a period of five years only those cases were followed by glial replacement and eventual registered as fluctuant hearing loss, who sclerotic plaque formation. Hearing loss does not themselves complained about it. Never a leading follow consistent patterns. Frequently the hearing question about fluctuant hearing loss was asked loss is of sudden onset, with a high incidence of from patients. After a careful detailed history all spontaneous