Clinical features and long-term outcome of ipsilateral delayed endolymphatic hydrops

BACKGROUND: Ipsilateral delayed endolymphatic hydrops (IDEH) is a disease characterized by recurrent acute vertigo that manifests after the appearance of a severe sensorineural non-hydropic hearing loss without symptomatology at the other ear. The cause of hearing loss can be congenital or acquired and, in the latter group, the hearing loss is usually sudden or secondary to head or acoustic trauma, ear surgery, viral infection, otitis media, streptomycin, meningitis, inner ear abnormality or idiopathic. METHODS: Data from 37 IDEH patients, who account for 3.4% of the overall sample of menieric patients at our institution, were analyzed in this retrospective study. RESULTLTS: The mean age of the sample was 58 years. Mean duration of the disease at the moment of diagnosis was 57 months. Follow-up ranged from 24 to 180 months (average: 87 months). Hearing loss at the ear cause of IDEH was due to sudden hearing loss in 23 cases (62%), chronic otitis operated on in five cases (14%) and congenital in one case (3%); in eight cases (21%) the cause of hearing loss was unknown. Mean age of appearance of IDEH was 54 years, mean interval between the appearance of HL and IDEH was 82 months and mean duration of the disease at the time of diagnosis was 57 months. Mean PTATA threshold at the affected ear was 88 dB. After dietetic and medical treatment, with a follow-up of 24 to 180 months (average: 87 months), we have obtained the resolution of vertigo crisis, or an acceptable reduction in crisis intensity and/or frequency, in 50 patients (88%). In the remaining seven patients intratympanic gentamicin, following the titration method, was applied, with the resolution of vertigo in six of them (88%); in one cases we have carried out the labyrinthectomy, that solved vertigo crisis. CONCLUSIONS: In conclusion IDEH is a not frequent form of Ménière's disease with good long-term prognosis and in case on vertigo not responsive to medical therapy intratympanic gentamicin labyrinthectomy can solve symptomatology.