Endolymphatic Sac Tumor as a Ménière-Like Vertiginous Syndrome: A Case Report.

Endolymphatic sac tumors are rare benign neoplasms with locally aggressive behavior located in the posterior petrous ridge of the temporal bone. They cause sensorineural hearing loss and may develop vestibular damage. A 24-year-old male patient arrived at our office with a history of acute vertiginous syndrome, left hearing loss, and tinnitus 1-year ago. His chief complaint was an increase in auditory symptoms. A CT scan and MRI showed an endolymphatic sac tumor. Complete resection of the lesion was achieved by a transmastoid and translabyrinthine approach. Low-grade adenocarcinoma was confirmed by histopathology. The patient remained without clinical vestibular symptoms. However, a small residual tumor was addressed by gamma-ray radiosurgery. Postoperative deep left sensorineural hearing loss was identified, without any vestibular sequelae. Radiologic imaging is the most useful tool for this diagnosis. Endolymphatic sac tumors should be in the differential diagnosis of recalcitrant audio-vestibular symptoms. Complete surgical resection is the most appropriate management.

Impact of bilateral nasal polyposis on the interoptic and interzygomatic distance.

PURPOSE: To identify and determine variations on eye distance in patients with bilateral nasal polyposis (BNP) compared to a healthy control group. METHODS: This is a case-control study that included 20 BNP patients and 40 healthy controls. We included all patients with BNP confirmed by pathology and a computed tomography scan. A healthy control group was admitted, filtered by the exclusion criteria of nasal polyposis, craniofacial malformations, and encephalocele. Paranasal sinus CT scans were performed in all participants, and two measures were evaluated, the interoptic (soft tissue) and the interzygomatic (bone structure) distances. RESULTS: A total of 20 BNP subjects, 13 (65%) male and 7 (35%) female, with a mean age of 38.8 years, and 40 healthy controls, 16 (40%) male and 24 (60%) female with a mean age of 43.2 years, were included. The mean interoptic distance was 69.7 mm (71.9 mm men, 66.4 mm women) and interzygomatic distance was 103.1 mm (104.5 mm men, 100.6 mm women). A significant increase of the interoptic (p < 0.001) and interzygomatic (p < 0.002) measurements was found in patients with polyposis compared to the controls. In the receptor operative curve analysis, the interoptic distance had an area under a curve of 96% and the threshold that maximizes the sensitivity and specificity was 59.85 mm (sensitivity 90%, specificity 95%, PPV 90%, NPV 95%). CONCLUSIONS: An increase in ocular and orbital distances was identified in patients with BNP. Polyposis may be identified by measuring eye separation. The established cut point distance identifies patients that may benefit from follow-up. Further research in this study line is suggested.

Early hearing loss detection in rheumatoid arthritis and primary Sjögren syndrome using extended high frequency audiometry.

The aim of this study is to evaluate the hearing behavior of rheumatoid arthritis (RA) and primary Sjögren syndrome (PSS) patients and compare them with a healthy control group and with each other. A comparative cross-sectional study was performed with a group of 117 female RA patients, a group of 60 female PSS patients, and a 251 female healthy control group. Every subject underwent a series of studies including high-frequency audiometry, speech audiometry, and tympanometry. The high-frequency audiometry measured 250 to 16,000 Hz. The 117 patients with RA and the 60 with PSS were diagnosed according to American College of Rheumatology criteria / ACR 2010, and the validated classification of the American-European Consensus Group. Hearing loss was present in 36.8% of the RA group in 500-3000 Hz, 68.4% in 4000-8000 Hz, and 94.9% in 10,000-16,000 Hz. Hearing loss was present in 60% of the PSS group in 500-3000 Hz, 70% in 4000-8000 Hz, and 100% in 10,000-16,000 Hz. The hearing impairment prevalence of both groups was significantly different (p < 0.05) when compared with the healthy control group. We also compared the hearing thresholds between RA and PSS patients, finding a significant hearing threshold increase in 500-3000 Hz of the PSS group. This study consolidates the association between RA and PSS with hearing impairment. A deeper hearing loss was reported in PSS than in RA patients, demonstrating a greater auditory and speech recognition repercussion.

Dyspnea and dysphagia associated to hypopharyngeal fibrolipoma: A case report.

Fibrolipomas are benign lesions conformed by fat and connective tissue, classified as histologic variants of lipomas. They are rarely located in the head and neck and represent less than 0.6% of the benign tumors of the larynx and hypopharynx. Their clinical presentation depends on its location and size. We present the case of a 51-year-old male patient who reported progressive dyspnea, dysphagia and obstructive sleep symptoms with a duration of 3 months, without apparent cause. A pharyngolaryngeal fiberoptic endoscopy showed a smooth, rounded mass in the posterior wall of the hypopharynx, partially obstructing the laryngeal vestibule, creating a valve effect. Complete trans-cervical resection of the lesion was performed after the airway was secured by means of a tracheotomy. The final histopathology report was fibrolipoma. He is currently asymptomatic and without evidence of relapse one year after the procedure.

[Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis]. / Hipoacusia neurosensorial subclínica en pacientes femeninas con artritis reumatoide.

BACKGROUND: The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. OBJECTIVE: To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. MATERIAL AND METHODS: Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. RESULTS: High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. CONCLUSIONS: Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies.

Acalasia, alacrima sin insuficiencia suprarrenal, con disfunción neurológica periférica y autonómica (síndrome de Allgrove) / Achalasia, alacrima without adrenal insufficiency, with pheripheral and autonomic neurological dysfunction (Allgrove's syndrome)

Objetivo. Presentar un paciente con síndrome de Allgrove y disfunción neurológica periférica y autonómica. Un caso no informado previamente en la literatura médica nacional. Antecedentes. El síndrome de Allgrove es una entidad patológica poco común caracterizada por acalasia, alacrima (ausencia de lágrimas) e insuficiencia suprarrenal el cual se presenta por lo general en niños. En la literatura se han descrito casos aislados. La serie más grande, consistente en 20 pacientes procedentes de varios países de Europa, fue publicada en 1993. Método. Se describe un caso clínico compatible con síndrome de Allgrove y se realiza un revisión bibliográfica haciendo énfasis en las alteraciones neurológicas las cuales no han sido bien caracterizadas en estos pacientes. Resultados. Se presenta un paciente de 12 años de edad quien fue admitido al hospital por sifagia. Se sospechó el diagnóstico de acalasia por endoscopia, esofagograma y se confirmó por manometría. El examan clínico mostró además alacrima y datos de disfunción neurológica y del intelecto. La prueba de estimulación suprarrenal con ACTH (Cortosin) fue normal. La evaluación neurológica mostró datos de neuropatía periférica de predominio motor y axonal; datos clínicos de neuropatía autonómica; afección de la vía corticoespinal y retraso psicomotor y del coeficiente intelectual. Conclusiones. El síndrome de Allgrove debe incluirse en el diagnóstico diferencial de los infantes con acalasia. La insuficiencia suprarrenal podría aparecer después de realizado el diagnóstico de la enfermedad. La disfunción neurológica parece ser la lesión más prominente de este síndrome