Injected 3T-3D-FLAIR-MRI labyrinthine patterns match with the severity and tonotopic alteration in sudden sensorineural hearing loss.

PURPOSE: The aim of the study was to assess a correlation between MRI labyrinthine changes detected with IV-gadolinium optimized high-resolution 3D-FLAIR sequences 4 h after injection (OPT4-3DFLAIR) and the type of SSNHL, in terms of frequency alteration and severity. METHODS: This was a prospective monocentric study achieved from July 2019 to December 2020. The inclusion criterion was acute hearing loss of at least 30 dB over three contiguous frequencies occurring within a 72-h period, documented by a pure-tone audiometry (PTA). The primary endpoint was the visual assessment of hyperintensity in labyrinthine structures on OPT4-3DFLAIR performed on 3T MRI. RESULTS: Thirty-six affected ears were included (20 men, 15 women; mean age: 54.5 ± 16.3 years) with 69.4% full-spectrum hearing loss. The median hearing loss, expressed as median and interquartile range [IQR] was 91 dB [74-120], with 47.2% of concomitant acute vestibular syndrome. Pathological signal was found in 26 out of 36 ears (72.2%). Basal turn enhancement was found in all abnormal MRIs, with 73.1% of apical turn enhancement and 50% of vestibular enhancement. Seventeen on 19 cases (89.5%) with apical involvement on MRI had low-frequency hearing loss. Vestibular involvement on MRI was significantly associated with a wider frequency range of hearing loss (p = 0.0002) and the severity of SSNHL (84.5 [71.7-92.5] dB versus 120 [85.8-120] dB, p = 0.0158). CONCLUSION: This report shows that in pathological MRI in SSNHL, a pathologic cochlear base signal is always detected, a cochlear apical turn enhancement matches with low-tone impairment, and a pathological signal within the posterior labyrinth is associated with an impairment of all frequencies and the severity of SSNHL.

Serpentine supravenous hyperpigmentation induced by the nitrosourea fotemustine.

Two cases of serpentine supravenous hyperpigmentation developing in the area of fotemustine infusions are reported. Histological features showed an increased melanin synthesis and the presence of melanophages without focal degeneration of basal cells or dermal inflammatory infiltrate. Perls' strain was negative. Hypotheses concerning the mechanisms of increased melanin synthesis over the veins are discussed.

Extraocular sebaceous carcinoma.

A 79-year-old man with no history of previous irradiation presented with a large ulcerated tumour of the cheek. The histological features favoured a true sebaceous carcinoma. Neither squamous nor basal differentiation were seen. Sudan stainings were positive. Tumour cells expressed suprabasal keratins and were negative for carcinoembryonic antigen, vimentin and S 100 protein. Extraocular sebaceous carcinomas occurring without previous irradiation are rare tumours which behave aggressively. Treatment regimens are discussed.

Letterer-Siwe disease and subacute monocytic leukemia.

We report a case of typical Letterer-Siwe disease associated with a subacute monocytic leukemia in a 75-year-old man. The simultaneous presentation of both diseases is rare. It may be speculated that our patient did not present a chance association of two different processes but a disorder of the Langerhans cell-mononuclear phagocytic system lineage.