Sensorineural hearing loss with delayed onset of vertigo☆, ☆☆, ★, ★★

1995 ◽  
Vol 112 (4) ◽  
pp. 540-543 ◽  
Author(s):  
A LANGMAN ◽  
R LINDEMAN
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Delayed Onset

scholarly journals Should You Follow the Better-Hearing Ear for Congenital Cytomegalovirus Infection and Isolated Sensorineural Hearing Loss?

2019 ◽  
Vol 162 (1) ◽  
pp. 114-120 ◽  
Author(s):  
Vanessa Torrecillas ◽  
Chelsea M. Allen ◽  
Tom Greene ◽  
Albert Park ◽  
Winnie Chung ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Early Onset ◽  
Medical Center ◽  
Antiviral Treatment ◽  
Sensorineural Hearing ◽  
Cmv Infection ◽  
Congenital Cytomegalovirus ◽  
Delayed Onset ◽  
Hearing Thresholds

Objective To describe the progression of sensorineural hearing loss (SNHL) in the better- and poorer-hearing ears in children with asymptomatic congenital cytomegalovirus (CMV) infection with isolated SNHL. Study Design Longitudinal prospective cohort study. Setting Tertiary medical center. Subjects and Methods We analyzed hearing thresholds of the better- and poorer-hearing ears of 16 CMV-infected patients with isolated congenital/early-onset or delayed-onset SNHL identified through hospital-based CMV screening of >30,000 newborns from 1982 to 1992. Results By 12 months of age, 4 of 7 patients with congenital/early-onset SNHL developed worsening thresholds in the poorer-hearing ear, and 1 had an improvement in the better-hearing ear. By 18 years of age, all 7 patients had worsening thresholds in the poorer-hearing ear and 3 patients had worsening thresholds in the better-hearing ear. Hearing loss first worsened at a mean age of 2 and 6 years in the poorer- and better-hearing ears, respectively. Nine patients were diagnosed with delayed-onset SNHL (mean age of 9 years vs 12 years for the poorer- and better-hearing ears), 6 of whom had worsening thresholds in the poorer-hearing ear and 1 in both ears. Conclusion In most children with congenital CMV infection and isolated SNHL, the poorer-hearing ear worsened earlier and more precipitously than the better-hearing ear. This study suggests that monitoring individual hearing thresholds in both ears is important for appropriate interventions and future evaluation of efficacy of antiviral treatment.

Sensorineural Hearing Loss with Delayed Onset of Vertigo

1995 ◽  
Vol 112 (4) ◽  
pp. 540-543 ◽  
Author(s):  
Alan W. Langman ◽  
Roger C. Lindeman
Keyword(s):  
Hearing Loss ◽  
Surgical Treatment ◽  
Sensorineural Hearing Loss ◽  
Clinical Experience ◽  
Sensorineural Hearing ◽  
Caloric Testing ◽  
Delayed Onset ◽  
The Past ◽  
Episodic Vertigo

Sensorineural hearing loss with delayed onset of vertigo is a syndrome in which episodic vertigo arises in a person who has preexisting unilateral severe-to-profound sensorineural hearing loss. This syndrome has an ipsilateral form in which the vertigo arises from the poorer hearing ear and a contralateral form in which the aural symptoms arise from the better hearing ear. The existence of this syndrome has only been noted within the past two decades. This report details our clinical experience with 17 persons with the ipsilateral form of this disorder. The onset of the vertigo after the occurrence of the hearing loss was quite variable. It ranged from 1 to 60 years after the development of the hearing loss. The hearing loss occurred for several reasons. Most patients had hearing loss due to an unknown cause. The development of the vertigo and the timing of the onset of the vertigo were not related to the cause of the hearing loss. Bithermal caloric testing identified the offending labyrinth in most patients who underwent ablative vestibular surgery. Ablative vestibular surgery was performed in 13 of the 17 persons in this study because of disabling symptoms. In all 13 cases, the episodic vertigo was eliminated. Surgical treatment for sensorineural hearing loss with delayed-onset vertigo, as with all surgery for vertigo, should be based on the severity of the afflicted person's symptoms.

scholarly journals Gangguan pendengaran pada sindroma LEOPARD

2012 ◽  
Vol 42 (2) ◽  
Author(s):  
Semiramis Zizlavsky ◽  
Ronny Suwento ◽  
Dina Alia
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Pulmonary Stenosis ◽  
Sensorineural Deafness ◽  
Sensorineural Hearing ◽  
Leopard Syndrome ◽  
Brainstem Evoked Response Audiometry ◽  
Delayed Onset ◽  
Ocular Hypertelorism ◽  
Evoked Response Audiometry

Background: Leopard syndrome is a rare case, only around 200 cases has been reported worldwide.  Leopard syndrome is abbreviation for multipel Lentigines, Electrocardiographic conduction, Ocular hypertelorism, Pulmonary stenosis, Abnormality of genitalia, Retardation of growth, and sensorineural Deafness. This disorder suggests a possible relation between PTPN11 gene mutations and distinct clinical features. Purpose: This case is presented so that ENT specialists could identify signs and symptoms of Leopard Syndrome which manifest as sensorineural hearing loss (SNHL). Case: We report a 29 year old woman with multiple lentigines, scoliosis and atrial septal defect. She has 4 year old twin boys, one of them has cryptorchidism and a 10 month old girl with asymmetric septal hypertrophy and they also have multipel lentigines. They were referred to ENT Department for auditory function screening since  Leopard syndrome is suspected. The audiometry of the mother reveals mild conductive deafness (40 dB)  in right ear due to tympanic membrane perforation. Audiometry of the twin boys reveals sensorineural hearing loss above 4000 Hz frequency. Brainstem Evoked Response Audiometry (BERA) of the daughter reveals mild sensorineural hearing loss (40 dB )on right ear. Genogram shows that the disorders is dominant autosomal inherited. Management: Periodic auditory examination for sensorineural hearing loss is recommended since delayed onset could occur. Conclusion: Sensorineural hearing loss is a mani-festation of Leopard syndrome that should be assessed early and periodically to detect delayed onset. Keywords: Sensorineural hearing loss (SNHL), Leopard syndrome, generalized lentiginosa.    Abstrak :  Latar belakang: Sindroma Leopard merupakan kasus yang jarang ditemukan dan dari publikasi yang ada, hingga saat ini hanya terdapat 200 kasus di seluruh dunia. Sindroma Leopard merupakan singkatandari Lentigines multipel, Electrocardiographic conduction, Ocular hypertelorism, Pulmonary stenosis,Abnormality of genitalia, Retardation of growth and sensorineural Deafness. Kelainan ini disebabkan olehmutasi gen PTPN11 dengan gambaran klinis yang khas. Tujuan: Kasus ini diajukan agar spesialis THTmengenali gejala sindroma Leopard yang dapat melibatkan gangguan pendengaran berupa sensorineuralhearing loss(SNHL) sehingga tidak terjadi keterlambatan dalam penatalaksanaan. Kasus: Perempuanberusia 29 tahun dengan lentiginosa multipel, skoliosis dan defek septum atrium. Ia memiliki 2 anaklaki-laki kembar yang salah satunya mengalami kriptorkismus dan anak perempuan usia 10 bulan yangmenderita hipertrofi septum asimetris dan juga menderita multipel lentiginosa. Mereka dikonsulkan ke THT dari bagian kulit RSCM untuk pemeriksaan fungsi pendengaran dengan kecurigaan sindromaLeopard. Pada pemeriksaan audiometri diperoleh hasil pada ibu berupa tuli konduktif ringan (40 dB)telinga kanan akibat perforasi membran timpani. Dua orang anak kembar menunjukkan hasil tuli sarafdi atas frekuensi 4000 Hz. Pemeriksaan Brainstem Evoked Response Audiometry (BERA) pada anakperempuan menunjukkan tuli saraf ringan (40 dB) di telinga kanan. Genogram menunjukkan kelainanautosom dominan. Penatalaksanaan: Tindak lanjut berupa pemeriksaan pendengaran secara berkaladianggap penting untuk mendeteksi terjadinya awitan lambat. Kesimpulan: SNHL merupakan salah satumanifestasi sindroma Leopard yang perlu diperiksa untuk mendeteksi terjadi awitan lambat. Kata kunci: Sensorineural hearing loss (SNHL), sindroma Leopard, lentiginosa multipel.

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