Czigner, Jenő

Janáky, Márta

Kiss, József Géza

László, A.

Szabados, É.

Kiss József Géza

Janáky Márta

László A.

Szabados É.

Czigner Jenő

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VERTIGO, NAUSEA, TINNITUS AND HYPOACUSLA DUE TO CENTRAL DISEQUILIBRIUMVISUAL MECHANISMS IN BALANCE CONTROLProceedings of the XIX th Scientific Meeting of the Neurootological and Equilibriometric Society (NES) Bad Kissingen - Germany, 27-29 March, 1992Volume XXEditors:Claus-Frenz ClaussenBad Kissingen, GermanyMüind V. KirtaneBombay, IndiaDieter SchneiderWürzburg, Germanymedicin + pharmacie dr. werner rudat & Co Nachf. edition m + p • Hamburg 1994Proceedings o f the NES, Volume XX, 1992C.-F. Claussen, M. V. Kirtane, D. Schneider, editors97A  STUDY OP CENTRAL COCHLEOVESTIBULAR FUNCTION IN AMAUROTIC CHILDREN KISS, J.G., JANÁKY, M.*, LÁSZLÓ, A.**, SZABADOS. É., CZIGNER, J.DEPARTMENTS OF OTORHINOLARYNGOLOGY, OPHTALMOLOGY* AND PEDIATRICS** SZEGED , HUNGARYKey word«: BAEP, central hypoxic optic lesion, optic nerve atrophy, retineal degeneration, Usher's syndromeSummary19 amaurotic infants and children were investigated using the acoustic evoked potentials (BAEP) method. Distribution o f the patients was:1. Central hypoxic optic lesion's group (n=6),2. Optic nerve atrophy (n=4),3. Retinal degenerations (n=5),4. Usher's syndrom (n=4).1. In the central hypoxic optic lesion's group, there were 4 cases with normal responses, 1 case with an unilateral retrocochlear lesion and another with a bilateral cochlear lesion.2. Among the children with optic nerve atrophy, 2 showed normal BAEP values, 1 had an unilateral retrocochlear and another a bilateral cochlear lesion.3. In the group o f retinal degenerations there were three families having genetic ground, 2 of them were X-linked recessive heredity, 4 cases had normal values, and 1 case proved to have a unilateral retrocochlear higher brainstem lesion.4. All 4 cases with Usher's syndrom showed uni- or bilateral cochlear lesions and 2 of them showed bilateral retrocochlear lesions too.According to our results in several types o f amaurosis the BAEP investigation have diagnostic values for a central (brainstem) affection.IntroductionThe previous BAEP investigations have been made in demyelinisation syndromes, multiple sclerosis, motor system diseases (1,7,8), acoustic neurinomas, brain tumors etc. (5).Infants and children suffering from different types of amaurosis were investigated by BAEP according to that for the evaluation o f differential diagnostic values.Patients and methods19 amaurotic infants and children were investigated by BAEP method. Distribution o f the patients was:1. Central hypoxic optic lesion's group (n=6),2. Optic nerve atrophy (n=4),983. Retinal degenerations (n=5),4. Usher's syndrome (n=4).Responses to 2 000 click presentations were sampled over a 10 ms time window.In the rate study, the click were presented at 13/s, with an intensity o f 80 dB SPL. The responses were recorded differentially between the vertex and the electrode fixed to the mastoid process o f the tested ear, with the contralateral mastoid process electrode serving as ground. The latencies of the waves (I,II,III and IV/V) and inter-peak latencies (IPLI-III, III-IV/V, I-IV/V) were detected and automatically analised by computer (3,4).The otoneurologic investigation consisted o f registration o f spontaneous vestibular symptoms, foveal optokinetic nystagmus and caloric vestibular reaction according to Cawthome- Fitzgerald-Hallpike method. The nystagmus was recorded electronystagmographically.ResultsClinical electrophysiological and neurological data, Interpeak latencies and BAEP curves are summarised In Table I., fig. la-b. and fig.2 a-b-c-d.The fig. la  represents BAEP peak absolute latencies in ms with SD, in the 4 investigated groups . It can be seen that on the 1st group: 6 cases , 12 ears - 9 ears had a normal BAEP pattern and two cases - one person both ears and another child one side- BAEP waves were abnormal.In the 2nd group (optic nerve lesion) we found 3 ears with abnormal BAEP pattern.In the 3rd group we found only one ears a delayed BAEP absolute latencies.In the last group all four patients had on both sides pathological evoked potentials (fig. 2a, b, c,d).Fig. lb  represents a normal value of the inter-peak latencies (IPL) In msec o f all 4 groups. In the 1st group (central optical hypoxic lesion) we found one case with one side delayed IPL III-IV/V and IPL I-IV/V indicating a retrocochlear lesion. In the 2nd group (Optic nerve lesion) also one was affected on one side from delayed IPL I-III and IPL I-V/V, related to a retrocochlear lesion too: against to the previous case, the retrocochlear lesion proved to be a lower brainstem region (at the level o f oliva superior). In the next group (retinal degeneration) we found one case and one side with a similar BAEP pattern to the previous case, it means a similar retrocochlear lesion (the I-III and the I-IV/V IPL were delayed). In the last group (Usher's syndrome) two cases had on both sides a pathological BAEP pattern in all IPL-s, indicating a more serious brainstem affection.(See next page!)Table 1.CLINICAL, ELECTRO PHYSIOLOGICAL AND NEUROLOGICAL DATANAM E AGE VEP ERG AUDIOGRAM BERA CT NEUROL.STGroup 1. Dg: Central hypoxic optic laesion (n=6)l.K .D . f  l l y ext. ext. u.l.RCh.l.d. norm. moscl .hypotoniavisuomot incoord2.Z.S. m 8y norm. - norm. norm. ■corp.calLagenessia no organic laesion3.L.B . m 8m ext. - norm norm. nystagm.norm.brain scint.4.T.S. f  l ly min.response norm. norm. norm norm. no organic laesion5.T .R . m 7m - - b.l.Ch. norm. chondrodyspl.mkroceph.6.B .K . f  10m min.response norm - norm norm. strabizmos, astasia-abasiaGroup 2. t)g : Optic nerve atrophy (n=4)l.C .H . m l3y ext. norm. - b.l.Ch. norm.2.M .M . f  14y ext. norm.macul.deg. norm norm.3.S.P. f  13y ext. cong. Leber amaurosisext. uul.RCh.Ld. norm. abnorm .in.,V. Ls.4.M .O . f  12y min.response fam ilial opt.atrophyext. norm. norm. norm.ao organic laesion no organic laesion no organic laesionno organic laesionm =m ale, f= fem ale, ext.= extinguished, Ch.= cochlear, RCh.=retrocochlear, u.l. «unilateral, b.l.=bilateral, norm.= normalCDCDTab. 1 -Group 3. Dg: Retineal degeneration (n=5)I.R .F . m 14y subnorm. norm. norm. norm. norm. borisontal nystagmusx-linked tapetoret deg. 2 J .K . m 12y photopic ext. norm. norm. norm. no organic laeskxtTapetoret. scotopk ERGdeg.aut.dom. subnorm.3.S.K. f  lOy photop.ERG ext. norm, norm. norm. norm. no organic laesionTapetoret. scotopic ERGdeg.aut.dom. subnorm.4.E.S. f  15y l.s.unregular min.resp.norm. u.l.Rch.l.s. norm. no organic laesionatypic ret. I.d.ext I.d.ext.deg.famiUal 5 .K .M . f  14y x-linkednorm. norm. norm. norm. norm. 17 D myop.cong.nyst.tapetoret.deg.Group 4. Dg: Usher sy.(n=4)I.L .R . m 5y l.s.ext. ext. b.l.Ch. cong. defn.l.d.min.resp deg. pigm. retinae (DPR)2.S.R. 4m l.s.ext. ext. u.l.Ch.I.s. CT.xortk.atrophy. n.optfe atrophy DPRl.d.min.resp deafn. mutism3.A .P . f  U y ext. - u.l.Ch.l.d. - dysbasia, ataxia, ment.retard.b.l.RCh. IQ.: <30,mutism, EIXJ low ampl.,DPR4.Z .P. m 3y ext. - b.l.Ch. cong. deafn. ment.retard.,b.LRCh. - mutism, DPRpsychomot.ret.EEG: theta-ddta act. beta superpos.rot.ayst.100101II III IV/V P E A Ky —+■ Cent.hypox. y —* Opl.nerv atr. Ret. deg.V - Usher sy.i-iii.in-iv/yi-iv/vI  P Ly —*. Cent.hypox. Y f-  »  Opl.nerv atr. Lk-*. Ret. deg.* Usher sy.Ic .M i lto wir r- f MAu ' 11 rrm M ^V î^—<rV-Ir**'----_. c m a »  a t .IMCVWSMb.cL102]  M.K. Wjjj /  9 « «  «• *e KgW by op«« - f a'wv— J Vr»—y»■■—■. V .....—----- — -----TvJV----\ * T  MIC * 1  t f  OKNA/Ayv/VvAv/V'%^104Discussion1st group. (Central hypoxic optic atrophy): Pathological VEP, normal ERG except K.D. (No=l) where both o f VEP and ERG were extinguished (Table 1. and fig 2.a). The pathological VEP means cortical damage o f the visual cortical region, and the normal ERG means the visual pathway were intact. Guimard et al. (2) have investigated auditory brainstem responses (BAEP) and ultrasound changes in a high-risk infants population. BAEP abnormalities were found in 40% o f the patients. The more extensive the cerebral hypoxic-ischaemic lesions were, the more severe the BAEP abnormalities were. The latter were o f audiological type in 14%, neurological abnormalities were rare (6%). They concluded, that ABR represents the method o f first choise for the detection o f hearing loss, in the Neonatal Unit, while it seemed to be o f limited value in assessing the brainstem function. We have similar results. All members o f this group were mature neonates, the perinatal hypoxic lesions were retrospectively detected during the pathomechanical investigation of the central optic atrophy.Vestibulometryrln the group with central hypoxic optic lesion with open eyes a pendular eye movement was found in 1 and eye-oscillation also in 1 subject. The optokinetic reactions were irregular. The caloric vestibular investigations revealed unilateral hypaesthesia in 2 patients and a marked nystagmus preponderance in an other.Und group (N. optic atrophy): In early childhood the ERG pattern are normal, later (depending on the age) will be progressively damaged.VEP were generally extinguished. Two cases (No:l&3 in Table 1 and fig 2b) proved to be Leber amaurosis: with pathological BAEP bilateral cochlear and unilateral cochlear laesion. Another two o f them had another type o f optic atrophy without any cochlear or retrocochlear disturbances. One o f the last two cases showed positive familial anamnesis, the father o f the 4th case suffered from DPRLeber's disease is a hereditary optic neuropathy linked to a mitochondrial DNA defect (9), with demyelinization o f the optic nerve. BAEP-s were anomalous in 64% o f patients without hearing defects (6).Illrd group (Retinal degenerations, in: Table 1 and fig 2.c): Two types o f  these have been identified: central and mixed. For the differentiation o f the above mentioned types photopic and scotopic ERG investigations are necessary. In the 1st and 5th cases x-linked tapetoretineal degeneration were detected, both o f them were affected boys. When the degeneration is a photopic type the ERG is extinguished indicating the cones are affected, while in scotopic type, the ERG pattern is subnormal but not extinguished, according to the degeneration o f the rodes. In case of No. 4 the retineal degeneration proved to be a mixed type, with the dominance o f the scotopic lesion. The BAEP showed unilateral retrocochlear lesion.The brother and sister o f the family K. proved to have autosomal dominant tapetoretinal degeneration (father was amaurotic too) with the disfunction o f the rods and cones.All member o f these group except No. 4 were audiologically intact.Vesibulometry: In the retinal degeneration's group spontaneus nystagmus was recorded in 2 patients only whit open eyes. The nystagmus was predominant on the side of eye abduction (fig.1053a-b). During optokinetic stimulation the slow phases o f the nystagmus were irregular. In 1 case pendular nystagmus non susceptible to optokinetic stimuli, was observed (fig. 4). The caloric reaction revealed unilateral vestibular hypoesthesia associated with nystagmus dysmetry in one patient, whereas the caloric response was totally missing in an other.P/th group (Usher's syndrome, in: Table 1 and fig. 2d): All o f them had an autosomal recessively inherited degeneratio pigmentosa retinae with disacusis syndrome, manifested in the same family in a brother and a sister (first cousins). These patients had variable serious degeneration both o f rods and cones with extinguished ERG patterns. VEP investigations gave only a minimal response or extinguished. In all cases o f these group were detected cochlear hearing loss and the P. sister and brother had an accessoriously different degree o f retrocochlear lesion.According to our results in several types o f amaurosis the BAEP investigation have diagnostic values for a central (brainstem) affection fTable 2).Table 2. Amaurotic children (n=19)Bera examinationsGr. Case Norm RCh.l. Ch.l.Centr.hypox.o.l. 6 4 1 u. 1. 1 b.l.Opt. nerve atr. 4 2 1 u.l. 1 b.l.Ret deg. 5 4 1 u.l. -Usher sy. 4 - 2 b.l. 2 u.l.+2 b.l.RCh.l. = retrocochlear lesionCh.l. = cochlear lesionu.l. = unilateralb.l. = bilateralAmong the amaurotic children in spite o f the vestibular lesions a spontaneous vestibular nystagmus was not recorded. The ocular nystagmus caused various disorders in the optokinetic responses. The vestibular lesions proved to be o f a central type. These were more marked in the cases o f retinal degeneration, but further investigations have to be carried out.Reference«1. Cascino, G.D., Ring, S.R., King, P.J.L., Brown, R.H., Chiapps, K.H.Evoked potentials in motor system diseases.Neurology 38, 231-238 /1988.2. Guinard, C., Fawer, C.L., Despland, P.A., Caleme, A.Auditory brainstem responses and ultrasound changes in a high-risk infants population.I Helv.paediat. Acts. 43, 377-388/1988.3. Jewett, D.L., Romano, M.H., Williston, J.S.Human auditory evoked potentials: possible brain stem components detected on the scalp. Science 167. 1517. /1970.1064. Kjaer M. ,Evoked potentials. With special reference to its diagnostic value.Acta Neurol. Scand. 61, 265. /1980.5. Mauerer K., Rochel M., Lowitzsch K.Early auditory evoked potentials: developmental aspects and validity in neuro-paediatric and audiologic disorders.Eur. J. Pediatr. 143, 13-17/1984.6. Mondelli M., Rossi A.BAEP changes in Leber's hereditary optic atrophy: futher confirmation o f multisystem involvement.Acta Neurol. Scand 81, 349-353/1990.7. Rossini P.M., Cracco J.B.Somatosensory and brainstem auditory evoked potentials in neurodegenerative system disorders.Eur. Neurol. 26, 176-188 /1987.8. Tackmann W., Vogel, P.Brainstem auditory evoked potentials evoked by clicks o f different polarity in multiple sclerosis patients.Eur. Neurol. 36. 193-198/1987.9. Wallace D.C., Singh G., Lott M.T., Hodge J.A., Schurr T.G., Lezza A.M.S., Elses L.J., Nikoskelainen, E.Mitochondrial DNA mutation associated with Leber's hereditary optic atrophy.Science 242, 1427-1430 /1988.

A study of central cochleovestibular function in amaurotic children

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A study of central cochleovestibular function in amaurotic children

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