SSDD may be the most common identifiable cause for PT from venous origin and is potentially treatable. Temporal bone CT scans should be included in a complete evaluation of PT.
Otitis media represents a broad spectrum of disease, which include acute otitis media and otitis media with effusion. As immunization with the pneumococcal conjugate vaccine has become more widespread, the microbiological landscape of otitis media has changed, which affects the treatment options facing clinicians worldwide. This review discusses the diagnosis and medical management of acute and chronic suppurative otitis media, the changes noted over the past decade, and briefly expounds on the surgical management of their severe complications.
Alström syndrome is a rare autosomal recessive ciliopathy caused by mutations in the ALMS1 gene. Hallmark characteristics include childhood onset of severe retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, and cardiomyopathy. Here we comprehensively characterize the auditory and otologic manifestations in a prospective case series of 38 individuals, aged 1.7-37.9 years, with genetically confirmed Alström syndrome. Hearing loss was preceded by retinal dystrophy in all cases, and had an average age of detection of 7.45 years (range 1.5-15). Audiometric assessments showed mean pure tone averages (0.5, 1, 2, 4 kHz) of 48.6 and 47.5 dB HL in the right and left ears, respectively. Hearing was within normal limits for only 8/74 ears (11%). For the 66 ears with hearing loss, the degree was mild (12%), moderate (54%), or severe (8%). Type of hearing loss was predominantly sensorineural (77%), while three ears had mixed loss, no ears had conductive loss, and type of hearing loss was indeterminate for the remaining 12 ears. Serial audiograms available for 33 patients showed hearing loss progression of approximately 10-15 dB/decade. Our data show that hearing loss associated with Alström syndrome begins in childhood and is a predominantly symmetric, sensory hearing loss that may progress to a severe degree. Absent otoacoustic emissions, intact speech discrimination, and disproportionately normal auditory brainstem responses suggest an outer hair cell site of lesion. These findings indicate that individuals with Alström syndrome would benefit from sound amplification and if necessary, cochlear implantation.
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