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Home , Conductive hearing loss, Otology, Vertigo

FLUCTUANT LOSS

S. K. Jaiswal Assistant Professor Department of , Nose and Throat S. K. Medical College Muzaffarpur, Bihar- 842003, India

122 cases of fluctuant were studied. Serous was commonest followed by Meniere's disease, Acoustic , fistula of round and oval window, oto-mandibular syndrome, Congenital and maxillary sinusitis. Cases of Arachnoid cyst, , 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 (ii) Congenital cholesteatoma. and . 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 . 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 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 . 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 is Meniere's disease. Hydrops This sensory-neural component (decreased of 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 (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 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 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 and its increased osmotic Labyrinthineg fistula. Mostly 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 and pathophysiology. We can not alter the Having the same symptoms as round window pneumatization of the , position of fistula aetiology of oval window fistula is different. sac and or perisccular fibrosis. So we have to Most common cause is post 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, 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 . 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 complete recovery. Recovery of pure the patients were subjected to complete Ear, Note tone thresholds occurs much before the recovery throat and T. M. joint examination. Tuning fork of speech discrimination (Citron et al 1963). test and was done in all cases. Cranial Abnormalities of Base of Skull and Cranio nerve and nervous system examination Cervical Region: Radiological examination viz. X-RAYS Mastoid, Abnormalities of base of skull and cranio PNS, Skull, cranio cerivical region were done in cervical region are inherited and rarely acquired. those cases who required these investigations. They are platybasia, basilar impression and dens After establishing the diagnosis along with axis extension into foramen magnum. A fully fluctuant hearing loss these cases were charted developed syndrome has symptoms and signs of out as shown in observation tables. IJO & HNS Special Issue, 1997 69 Fluctuant Hearing Loss--S. K. Jaiswal OBSER VA TION As shown in different tables TABLE 1 Total number of cases of fluctuant hearing loss- 122 Total No. of Fluctuant conductive Fluctuant sensory. Fluctuant neural cases hearing loss hearing loss hearing loss 122 86 32 4 70.50% 26.23% 3.27%

TABLE 2 Fluctuant conductive hearing loss-86 Total no. of cases Serous otitis media Oto-mandibular syndrome 86 84 02 97.67% 2.33%

TABLE 3 Fluctuant conductive hearing loss Distribution of serous otitis media in different age group-84

Total no. Age Group Age Group Age Group Age Group Age Group Age Group of cases 0-10 yrs. 11-20 21-30 31-40 41-50 51 -Onwards 0-5yrs 6-10 yrs yrs yrs yrs yrs yrs 84 7 33 25 11 6 2 Nil 8.3% 39.28% 27.76% 13.9% 7.14% 2.38%

TABLE 4 Fluctuant sensory hearing loss : 32 Total No. of Round window Oval window,Meniere's Cervical Septic foci cases fistula fistula disease Rib. Maxillary sinusitis

32 2 2 27(84.37%) Nil I doubtful Post Male / Female Barotrauma Stapedec 9 18 tomy 6.25% 6.25% 3.12%

IJO & HNS Special Issue, 1997 70 Fluctuant Hearing Loss--S. K. Jaiswal TABLE 5 Fluctuant neural hearing loss - 4

Total Acoustic Congenital Arachnoid Multiple Abn. base of No. of cases Neuroma Cholesteatoma Cyst Sclerosis Skull & cranio -cervical region 4 3 1 Nil Nil Nil 75% 25%

TABLE 6 Total No. of Cases - 122 Distribution of different diseases and their percentage Total no. Serous Meniere's Acoustic Fistulaof Fistulaof Oto- Congenital Maxillary of cases Otitis Disease% Neuroma% Round Oval mandibular Cholestea Sinusitis% Media % Window % Window % Syndrome % toma %

122 84 27 3 2 2 2 1 1 68.85% 22.13% 2.24% 1.63% 1.63% 1.63% 0.81% 0.81%

DISCUSSION Only single cases of congenital cholesteatoma In this present study of 122 cases of fluctuant and maxillary sinusitis each being 0.81% were hearing loss the fluctuant conductive hearing loss recorded with fluctuant hearing loss, showed their being the commonest 70.48% as against the rarity. Cases of Arachnoid cysts, multiple sclerosis findings of D. Plester (1982) in which he found and abnormalities of base of skull and cranio- inner ear fault in great majority of cases. In cervical region could not be presented in this fluctuant hearing loss, serous otitis media being series, probably because author might not be able the commonest 68.85%, this might be due to the to diagnose these cases at an early stage of fact that climate of North Bihar (India) is humid otological presentation or actually patients of these and damp, leading to upper respiratory tract abnormalities have not presented to him. In his inolvement in much greater percentage. As serous further study a delicate care will be taken to look otitis media is more common in younger age for these disease entity too. group, in this series too serous otitis media with To elucidate the cause a thorough investigation fluctuant conductive hearing loss was more is required including otological, neurological and commonly prevalent in younger age group. radiological examinations. In great majority of The second most common disease with cases inner ear is at fault (D. Plester-1982), which fluctuant sensory hearing loss in the present study may or may not have trigger mechanism else was Meniere's disease 22.13% coincides with the where having adverse influlence on the inner ear findings of many scientists. e.g. septic foci else where (Infected opaque sinus) cervical rib etc. But in this series middle ear fault Fluctuant hearing loss in Acoustic neuroma was found to be more common. was recorded upto 2.45%, in fistula of round window 1.63%, in fistula of oval window 1.63%, SUMMARY and in oto-.mandibular syndrome 1.63%, showed Fluctuant hearing loss is a common clinial finding. that these are not common. It has its genesis either in middle ear or inner ear IJO & HNS Special Issue, 1997 7: Fluctuant Hearing Loss--5. K. Jaiswal or in their central connections. A combined components. Endolymphatic hydrops or Meniere's middle ear and inner ear can ,occur as disease provide classical picture of fluctuating a consequence both stable and non-stable sensory neural hearing loss. A small percentage components can operate simultaneously. Middle of Acoustic neurOma, Congenital cholesteatoma, ear effusion, oto-mandibular syndrome, fistula of demonstrate a reversible neural hearing loss. oval and round window may some time show both

REFERENCE 1. Arnold, W. & Ganzer, U. (1980): Seromuco-tympanum and bone conduction: Proposed new classification of ear diseases caused by Eustachian tube dysfunction. In physiology of Eustachian tube and Middle ear (International Symposium, Freiburg, 1977) pp 71-78 p. 194 Georg Thieme verlag. 2. Citron, L., Dix, M., Hallpike, C., and Hood, J. (1963): A recent Clinico-pathological study of Cochlear nerve degeneration resulting from tumor pressure and disseminated sclerosis, with particular reference to the finding of normal threshold sensitivity for pure tones. Acta Otolaryngologica (Stockholm) 56, 330-337- as quoted by D. Plester. 3. D. Plester- (1982): Fluctuant hearing loss. -Edited by Alan G. Gibb and Mansfield F. W. Smith. Published by Butterwroths, International Medical Reviews-London-216:225. 4. Huizing, E. H. (1964): As quoted by D. Plester. 5. Jahnke, K. (1977): As quoted by D. Plester. 6. Klockhoff, I. and Westerberg, C. E. (1973): As quoted by D. Plester. 7. Munker, G. (1980): As quoted by D. Plester. 8. Schuknecht, H., Benites, J., Beekhuis, J., Igarashi, M., Singleton, C., and Ruedi, L. (1962): Pathology of sudden deafness Laryngoscope, 72, 1142-1157. 9. Scott-Brown-Disease of the Ear, Nose and Throat-3rd Ed. Vol. 2 Butterworths-London.

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