Portuguese Version

Year:  2004  Vol. 70   Ed. 3 - (17º)

Relato de Caso

Pages: 405 to 408

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Ménière's disease in children

Author(s): Gustavo J. Magalhães 1,
Gustavo G. Pacheco 1

Keywords: vertigo, Ménière's disease, treatment

Abstract:
The Ménière's Disease was described at first time by Prosper Ménière in XIX century and nowadays continues without explications for the etiology and fisiopatology. The disease has four symptoms that are correspond to: tinnitus, vertigo, hearing loss and aural fullness. The ages that this disease is most frequent are the third and the five decades of live and the occurrence in children is rare. This article want to show to the otorhinolaryngologists the existence of the Ménière's disease in child and the conduction of the case. In this case report a child with 11 years old presented as initial manifestation a facial edema and several cocleo-vestibular symptoms that was studied and confirm the diagnosis of Ménière's Disease. The treatment with labirintic depressive drugs promotes a good response.

INTRODUCTION

Vertigo is one of the most complicated symptoms for physicians and patients, since it is a difficult to describe symptom 1.

The American Academy of Otorhinolaryngology and Head and Neck Surgery defined as diagnostic criterion for Ménière's disease two or more episodes of vertigo that last equal or over 20 minutes; hearing loss documented at least once and presence of tinnitus and ear fullness 2.

The etiology of Ménière's disease is unknown and the autoimmune response is suggested as a possible pathogenesis of the disease, and there may be sudden sensorineural loss, such as in Cogan's syndrome and in Ménière's disease.

Histopathology is characterized by endolymphatic hydropsis with distension and distortion of membranous labyrinth produced by the endolymph 2, 5.
The reduction of absorption of endolymph by the endolymphatic sac and/or excess of endolymph production or even osmotically active proteins by the endolymphatic sac may be the genesis of this disease 2.

Charles Frazier, in 1908, was the first one to cure vertigo of Ménière's disease by sectioning the 8th cranial nerve 4. Normally the diagnosis is suspected by the clinical history and confirmed by complementary exams 1, 2.

LITERATURE REVIEW

Ménière's disease was described for the first time by Prosper Ménière in 1861 and we still have no definite explanation about its etiology and pathophysiology 4.

The disease may manifest by cochlear-vestibular symptoms, only vestibular or only cochlear symptoms and affections responsible for causing they can be known or not. Among the most well known causes we can list allergy, Lues disease, diabetes and trauma. The causing agent can be identified in about 50% of the cases 6.

In 1938, Cirus & Halpike described hydropsis as an increase in endolymph with dilation of membranous labyrinth owing to increase in production or reduction of absorption 2, 4, 6.

Anti-HSP70 antibodies were identified in the serum of patients with Ménière's disease. These antibodies were found in 33.3% of the unilateral cases and in 58.8% of the bilateral cases and reinforced the idea of autoimmune disease, but they are not clinically efficient for diagnosis 2. Allergic reaction type III changes the morphology and auricular electrophysiology, similarly to what is triggered by Ménière's disease 1.

The disease affects the most on the 3rd to 5th decades of life, being that the occurrence is rare in children. Mizukoshi et al., in a review of 7,418 cases of Ménière's disease in 1979 to 1999 had incidence of 1.5% of cases in children 7, being that its countrymen Akagi et al. obtained higher values in their study, demonstrating incidence of 2.9% of the total patients with vertigo 8.

In a typical crisis of Ménière's disease we can find rotation vertigo, hearing loss (in general, unilateral), tinnitus, aural pressure, discomfort to loud sounds and neurovegetative symptoms. Crisis can last minutes or hours. In-between crises there is improvement of symptoms, including hearing and tinnitus, intervals may last years or months and become closer and closer as the disease progresses.

Crises initially affect only low frequencies and later high frequencies as well. After some crises, all frequencies are affected and the hearing loss becomes irreversible, which can be identified by pure tone audiometry 8.

The occurrence of crises with fluctuation of hearing characterizes Ménière's disease. The diagnosis is made by history and confirmed by complementary tests 6. The audiometric pattern has been discussed and supraliminary tests determine the presence of Metz recruitment and absence of stapedial reflex with conduction hearing loss 4.

In the glycerol test, the patient has intake of dehydrating agent and there is increase in hearing confirmed by pure tone audiometry 2, 6.

Auditory brainstem response demonstrated difference in waves I and V, similarly to regular ABR. Electrocochleogram may indicate hydropsis, and it is a good test in these cases. We can collect few data if the patient is not in crisis 6.

The treatment can be clinical or surgical. Clinical treatment can be made during the crisis or in-between them 1, 6, 8. During the crisis, we can use labyrinthic depressors such as diphenidol, dimenidrinate, vitamin B6, associated to other drugs such as diazepinic, diuretic and vasodilators. It is recommended rest in bed, bland meals and no smoking, coffee or other labyrinthic stimulators. In the inter-crisis period, labyrinthic sedatives are administered for long periods of time 6.

Surgical indication comes into play when the clinical treatment is not satisfactory. There are two general types of surgical procedures: conservative and ablative. Conservative procedures comprise surgery of endolymphatic sac and cochleosacculotomy. Ablative procedures include labyrinthectomy, section of vestibular nerve, labyrinthic perfusion with streptomycin or middle ear instillation with gentamycin or streptomycin 9.
Differential diagnosis with acoustic neurinoma should be considered, in addition to vestibular neuritis, benign paroxysmal positional vertigo and Lermoyez syndrome 4.

CASE REPORT

J. M., aged 11 years, male, born in Queimadas - PB and living in Campina Grande - PB, came to the Center of Otorhinolaryngology, Hospital da Lagoa - RJ, complaining of marked tinnitus, sudden onset vertigo, hearing loss on the right, which had started in November 2001.

The symptoms appeared after one episode of sudden onset facial edema, which was seen in the state of Paraiba, where they considered the diagnostic hypothesis of acute renal failure. However, laboratory tests and complete blood count conducted during the facial edema episode demonstrated normal results, the same applying to urea and creatinine. Abdominal ultrasound of the kidneys showed topical and normal volume organs, ruling out the hypothesis of renal failure or nephrotic syndrome.

After the onset of clinical presentation in November 2001, the patient presented episodes that got worse, with marked rotation vertigo, vagal manifestations, tinnitus, ear fullness and reduction of hearing acuity on the right after each new crisis.

To start investigating, we ordered pure tone audiometry and vocal discrimination, in addition to impedanciometry. Audiometry showed decrease in pure tone thresholds on the right with mixed hearing loss, ranging from moderate to severe and affecting all frequencies, more marked in low frequencies. Impedanciometry demonstrated absence of stapedial reflex in frequencies of 1000Hz, 2000Hz and 4000Hz.

ABR detected absence of wave I on the right and normal latency of waves III and V, highly suggestive of cochlear lesion.

Vectoelectronystagmography was within normal range since the patient was not in the crisis during its execution.

To learn more about the renal failure we ordered urea, creatinine and fast glucose but they were all within the normal range.

To confirm the diagnosis of Ménière's disease we conducted the glycerol tests, in which we detected improvement of 15dB in frequency of 1000Hz and 10dB in frequencies of 500 and 2000Hz, confirming the diagnostic hypothesis of Ménière's disease.

To have differential diagnosis with neurinoma and inner ear congenital affections we ordered computed tomography that showed normal inner ear and internal acoustic canal structures.

Once defined the diagnosis, the patient started using dimenidrinate associated with B6 vitamin and cinarizine, since he had a crisis right after the end of investigation. After clinical treatment for one week, dimenidrinate and vitamin B6 were discontinued and cinarizine was maintained and the patient presented suppression of crises and stabilization of clinical presentation and audiometric pattern in a new audiometry.

DISCUSSION

Ménière's disease is still today a disease with etiopathogenesis to define. The initial clinical picture of facial edema presented by the patient that was the starting point of symptoms is undoubtedly the key to finding the etiology.

However, there are many different causes of facial edema in children. Among them we can include erysipela, allergic pictures, complicated rhinosinusitis or renal syndromes such as GNDA, nephrotic syndrome, and many others.

The initial hypothesis of acute renal failure or nephrotic syndrome was ruled out, and heart assessment did not reveal any pathology that justified facial edema. A possible allergic trigger could have caused the edema and would have justified the progression to Ménière's disease. However, the real etiology could not be confirmed, joining the 50% of the cases in which etiological diagnosis cannot be determined.

Another factor that attracts the attention in this case is the age range of the patient, since the most affected age ranges in Ménière's disease range from 3rd to 5th decades of life, being that the occurrence in children and adolescents is rare.

Clinical treatment promoted significant improvement in clinical presentation by putting an end to episodes that were very frequent, demonstrating that the use of vitamin B6 associated with dimenidrinate together with cinarizine promoted completion of the acute stage and isolated use of cinarizine maintained the patient in a stable presentation.

CLOSING REMARKS

Adolescence is not a period in which Ménière's disease is commonly found and inner ear congenital affections should always be considered in such occasions, being that differential diagnosis can be defined with CT scan or MRI.

To confirm the diagnosis of Ménière's disease, a high level of suspicion is required since only the association of many different symptoms presented by the disease together with a battery of complementary exams allow the diagnosis the disease, and specific exams such as glycerol test are indispensable for diagnostic conclusion.

REFERENCES

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8. Rauch SD, Zurckowski D, Block KJ. Anti-Heat Shook Protein 70 antibodies in Ménières' Disease. Laryngoscope 1999; 110:1516-21.
9. Rivas J, Guzman J.E. Combined Vestibular neurectomy and endolymphatic sac shunt via the retrosigmoid approach in the treatment of Ménière's Disease. Nose & Troat Journal 2001; 79 (8):571-5.
10. Silverstein H, Norell H. Retrolabyrinthine Vestibular Neurectomy. Otoryngol Head Neck Surg 1982; 90:778-2.
11. Tirzaman O, Facer G.W, Fabry D.A, Beatty C.W. Use of methotrexate for autoimmune hearing loss. Ann Otol Rinol Laryngol 2000; 109:710-4.

1 Resident Physicians, Service of Otorhinolaryngology, Hospital da Lagoa.
Affiliation: Hospital da Lagoa - RJ
Address correspondence to: Gustavo Jorge Magalhães - Rua Faro 19, ap.307 Jardim Botânico Rio de Janeiro RJ 22461-020
Tel (55 21) 2513-7144

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