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A CASE OF VESTIBULAR MONDINI WITH BPPV AND MENIERE'S DISEASE-LIKE SYMPTOMS

Chie Maekawa, Tadashi Kitahara, Arata Horii, Takeshi Kubo

Department of Otolaryngology, Osaka University, Suita, Japan

■ BACKGROUNDS: It is relatively difficult to diagnose patients with Mondini anomalies without CT or MRI, because symptoms in patients with Mondini anomalies are actually various due to the individual inner ear condition. In the present paper, we would like to demonstrate a rare case with labyrinthine anomaly with vertigo without cochlear symptoms, i.e. vestibular Mondini.

■ CASES: A 55-year-old male, a security guard in the building, attended our hospital, complaining of transient but persistent positioning vertigo in 2004. When he turned over right or left in his sleep, he felt transient vertigo and such evoked-symptoms were continuous more than one month and repeated once per a few months. Although there were no remarkable findings in audiogram, the apogeotropic positioning nystagmus was observed in spine position. This case was diagnosed as lateral semicircular canal (LSCC) type of BPPV with cupulo-lithiasis. We successfully performed Lempert maneuver for both directions a couple of times for one week.In 2005, he came to feel episodic vertigo continuously for 30 minutes. It appeared like Meniere’s disease without cochlear symptoms, i.e. vestibular Meniere’s disease. We examined CT and 3DMRI, indicating the hypoplastic L-SCC fused together with enlarged vestibule on the left side. We finally diagnosed that this case was affected with Mondini anomaly on the left side. Furosemide administration transiently improved the maximal slow phase eye velocity in the left ear, indicating endolymphatic hydrops on the same side with Mondini anomaly. The case was successfully treated with osmotic diuretics for one month.

■ CONCLUSIONS: Ohtani-I et al. clearly demonstrated with the temporal bone section of DiGeorge’s syndrome that hypoplastic LSCC was fused together with enlarged vestibule (Ann Otol Rhinol Laryngol, 1984). This finding suggests that it is relatively easy in such cases for the otolith in deformed utricle to be transferred into the fused space with vestibule and L-SCC, resulting in the irrigation of the hypoplastic cupula. The patient was obliged to work so hard as a security and came to feel stressed around the onset of episodic vertigo. Such a change of life style of the patient might be one of the reasons for endolymphatic hydrops.