Intracochlear Hemorrhage: A Rare Cause of Sudden Sensorineural Hearing Loss.

Inner ear hemorrhage is an extremely rare cause of sudden sensorineural hearing loss with few cases reported in the literature. We report the case of a 30-year-old male who presented with a sudden left ear hearing loss, with no tinnitus nor vertigo. The audiogram revealed a profound left sensorineural hearing loss. An MRI of the brain and internal auditory canal was performed 3 weeks after and revealed an increased signal intensity on T1-weighted (T1W) and T2 fluid-attenuated inversion recovery (FLAIR) images in the left cochlea. No other abnormalities were found, in particular no enhancement after intravenous administration of gadolinium. The CISS 3D sequence showed a signal of discreetly lower intensity in the left cochlea compared to the right one. The diagnosis of intracochlear hemorrhage was made. No improvement of the hearing loss has been noted after medical treatment and hyperbaric oxygen therapy.

Pseudo-Meigs' Syndrome in Tunisian H Syndrome Female Patient: First Case Reported.

H syndrome is an extremely rare autosomal recessive affection caused by biallelic mutations in the SLC29A3 gene encoding the human equilibrative nucleoside transporter hENT3. The hallmark signs are cutaneous consisting of hyperpigmentation and hypertrichosis patches. Besides, associated systemic manifestations are highly various reflecting phenotypic pleiotropism. Herein, we report a first case of pseudo-Meigs' syndrome occurring in a young Tunisian H syndrome diagnosed patient with a novel homozygous frameshift mutation in exon 2 of the SLC29A3 gene: p.S15Pfs*86 inducing a premature stop codon. The patient developed ascites associated with left ovarian mass and she underwent surgery. After tumor resection, ascites disappeared rapidly. Histological examination showed serous cystadenoma of the ovary orienting the diagnosis towards pseudo-Meigs' syndrome.