Thursday, October 28, 2010

Daily Short-Term Intratympanic Dexamethasone Treatment Alone as an Initial or Salvage Treatment for Idiopathic Sudden Sensorineural Hearing Loss

Audiol Neurotol 2011;16:191-197 
Seiji Kakehata, Akira Sasaki, Kazunori Futai, Rei Kitani, Hideichi Shinkawa
Department of Otorhinolaryngology, Hirosaki University School of Medicine, Hirosaki, Japan


Objective: Intratympanic (IT) steroid therapy has been proposed as an alternative treatment option for patients with idiopathic sudden sensorineural hearing loss (ISSNHL). However, the number and frequency of IT treatments and drug delivery methods remain to be determined. The purpose of this study was to evaluate the efficacy of daily short-term IT dexamethasone (DEX) treatment alone in ISSNHL patients using laser-assisted myringotomy (LAM) for the drug delivery route as an initial and/or salvage treatment. 

Study Design: Retrospective study. 

Setting: University hospital. Patients: Seventy-six ISSNHL patients receiving IT DEX. Patients with low-tone hearing loss, unilateral or bilateral fluctuating hearing loss or contralateral hearing loss were excluded. 

Intervention: DEX (4 mg/ml) was injected through a perforation made by LAM. IT DEX administration was performed on 8 sequential days. 

Main Outcome Measures: Pre- and postprocedure hearing levels. The average hearing level was determined by 5 frequencies (250, 500, 1000, 2000 and 4000 Hz). 

Results:Nineteen out of 76 patients fit the criteria for initial treatment in the study (group I), while 24 patients, who had failed systemic therapy, received salvage treatment (group S). The mean age of the patients in groups I and S was 56.2 years with a range from 31 to 73 years of age and 46.0 years with a range from 11 to 76 years of age, respectively. The mean number of days from onset of symptoms to IT therapy in groups I and S was 4.8 days with a range of 1–23 days and 15.3 days with a range of 6–28 days, respectively. In group I, 18 of the 19 patients (95%) showed improvement of more than 10 dB in the pure-tone audiogram, with a mean improvement of 40 dB. Twelve patients (63%) recovered completely and 16 patients (84%) demonstrated successful results with an improvement of more than 30 dB. In group S, 14 of the 24 patients (58%) showed improvement of more than 10 dB with a mean improvement of 16 dB. Two (8%) of the 7 patients (29%) with successful results recovered completely.

Conclusions: Daily short-term IT DEX administration using LAM for ISSNHL patients without concurrent therapy showed a high response rate and high cure rate and proved to be an alternative therapeutic option to high-dose systemic steroids as a first- and/or second-line treatment.

Copyright © 2010 S. Karger AG, Basel

A Novel Dominant and a De Novo Mutation in the GJB2 Gene (Connexin-26) Cause Keratitis-Ichthyosis-Deafness Syndrome: Implication for Cochlear Implantation

Otology & Neurotology:

Arndt, Susan; Aschendorff, Antje; Schild, Christian; Beck, Rainer; Maier, Wolfgang; Laszig, Roland; Birkenhäger, RalfFebruary 2010 - Volume 31 - Issue 2 - pp 210-215


doi: 10.1097/MAO.0b013e3181cc09cd

Objective: Keratitis-ichthyosis-deafness (KID) syndrome is a rare congenital disorder, characterized by hyperkeratosis and erythrokeratoderma associated with profound sensorineural hearing loss. Additional concomitant phenomena of the KID syndrome are dystrophic nails, dental abnormalities, scarring alopecia, and vascularizing keratitis. The disorder is caused by mutation in the GJB2 gene (connexin-26), a gap junction protein. The aim of this study was to explore the feasibility and procedure of cochlear implantation in patients with KID syndrome and to assess the genetic causes.
Study Design: Retrospective case review.
Setting: Tertiary referral center. Cochlear implant program.
Patients: We report on 2 cases of KID syndrome with congenital profound hearing loss. A 50-year-old woman with skin necrosis and implant extrusion 5 years after cochlear implantation and a 10-month-old infant girl with bilateral deafness, alopecia, bright light sensitivity, and congenital dermatosis.
Intervention: Genetic analysis. Cochlear implantation.
Main Outcome Measures: Mutation analysis, surgical suitability, and hearing rehabilitation.
Results: We detected a novel heterozygous missense mutation (Ile30Asn) in Patient 1 and a de novo mutation (Asp50Asn) in theGJB2 gene (connexin-26) in Patient 2. To decrease the risk of skin flap necrosis, we describe alternative surgical cochlear implantation techniques with a novel very thin receiver/stimulator (Nucleus CI 513; Cochlear Corp.). The postoperative course of both patients has been without any problems until now.
Conclusion: The combination of the cutaneous lesions with visual and auditory impairment demands to diagnose impaired hearing as early as possible. It would be helpful to search for KID syndrome in dealing with patients with deafness, skin lesions of unknown cause, and wound healing problems to choose the right method of surgical treatment and subsequent aftercare.

Ontogeny of Odor Liking during Childhood and Its Relation to Language Development

Chem. Senses (2010)doi: 10.1093/chemse/bjq101
  1. Fanny Rinck
  2. Melissa Barkat-Defradas
  3. Amandine Chakirian
  4. Pauline Joussain
  5. Fanny Bourgeat
  6. Marc Thévenet
  7. Catherine Rouby and  M
  8. oustafa Bensafi

Abstract
One important aspect of odor hedonics is its plasticity during human development. 
The present study set out to probe the modulators of such olfactory change during that period by testing the hypothesis that language and semantic representations of objects are strong organizers of odor liking. To this end, 15 three-year-old children were tested in a longitudinal study. Participants were exposed to exactly the same 12 odorants once a year over a 3-year period. At each experimental session, they were asked to answer 2 questions: 1) “Do you like or dislike this odor?” and 2) “Can you tell me what it is?” The level of language production was assessed on a standardized test. The 3-year-old children were found to categorize the same number of odorants as liked and as disliked. The follow-up study, in contrast, showed that at 5 years of age they categorized more of these odors as liked and that the shift was significant only in the children with higher language production skills. Taken as a whole, these findings suggest that the 3- to 5-year age range, when children begin to master language, is a turning point in the construction of olfactory hedonic categories during childhood.

Sunday, October 17, 2010

Posttonsillectomy hemorrhage: Blame on surgeons or genes?

The Laryngoscope

Volume 120Issue 9pages 1784–1787September 201
  1. Diana Arweiler-Harbeck MD, 
  2. Ender Öztürk MD, 
  3. Agnes Bankfalvi MD, PhD, 
  4. Stephan Lang MD, PhD, 
  5. Winfried Siffert MD, PhD,
  6. Kurt Werner Schmid MD, PhD, 
  7. Jürgen Peters MD, PhD, 
  8. Michael Adamzik MD, PhD

Abstract

Objective:

To investigate whether the insertion/deletion polymorphism (−94ins/delATTG) in the promoter of NFκB1 is associated with the risk of bleeding after tonsillectomy.

Design and Setting:

Retrospective study with genotyping performed from tonsillar tissue or blood.

Patients:

One hundred forty-eight patients having undergone tonsillectomy due to chronic tonsillitis, with or without posttonsillectomy hemorrhage.

Measurements and Results:

DNA-extraction from paraffin-embedded tonsillectomy tissue or blood was followed by genotyping for the insertion/deletion (−94ins/delATTG) promoter NFκB1 polymorphism. Genotypes differed significantly between patients with (n = 56) and without (n = 92) posttonsillectomy hemorrhage, with the frequency of the homozygous deletion genotype carriers (DD) significantly increased in those with posttonsillectomy bleeding with an odds ratio (OR) for bleeding of 3.78 (95% confidence interval [CI] 1.2–11.7, P = .023) but not in homozygous (II) insertion and heterozygous (ID) genotype carriers (II/ID). Genotype distribution in patients was compatible with the Hardy Weinberg equilibrium. In contrast, there were no statistically significant differences between patients with or without posttonsillectomy hemorrhage with regard to demographic characteristics, different surgeons, postoperative medications like analgesics, antibiotics, anticoagulation therapy, or values of variables of pre- and postoperative coagulation studies. Likewise, these variables revealed no differences between genotypes.

Conclusions:

Carriers of the homozygous deletion allele were at an almost fourfold risk to develop posttonsillectomy hemorrhage compared to homozygous and heterozygous insertion allele carriers, independent of other risk factors. Laryngoscope, 2010

Friday, October 15, 2010

Xylitol as a prophylaxis for acute otitis media: systematic review

International Journal of Audiology 49 (10), 754-61 (Oct 2010)
Danhauer JL, Johnson CE, Corbin NE, Bruccheri KG 


A systematic review was conducted to evaluate evidence regarding xylitol, a sugar alcohol, as a prophylaxis for acute otitis media (AOM) in children. The authors searched PubMed and other databases to identify evidence. Criteria for included studies were: appear in English-language, peer-reviewed journals; at least quasi-experimental designs; use xylitol; and present outcome data. The authors completed evaluation forms for the included studies at all phases of the review. 

The authors reviewed 1479 titles and excluded 1435. Abstracts and full texts were reviewed for the remaining 44; four randomized controlled trials met inclusion criteria. Xylitol was a generally well accepted prophylaxis for AOM with few side effects when administered via chewing gum or syrup at 10 g/day given five times daily. Meta-analysis revealed significant treatment effects (Risk ratio = 0.68; 95% confidence interval = 0.57 to 0.83). 

Xylitol can be a prophylaxis for AOM, but warrants further study, especially of vehicles other than chewing gum for young children, and information is needed regarding cost, duration of administration required, and expected long-term effects.

Hypothyroid-associated sensorineuronal deafness

Irish Journal of Medical Science (Sep 2010)
Comer DM, McConnell EM; 

A case of panhypopituitarism with hypothyroid-induced deafness in a man improving with hormone replacement is reported. 

A review of the medical literature reveals conflicting evidence regarding the underlying mechanism and prognosis of the defect in this context, but the association with hypothyroidism is more than spurious. 

Sensorineuronal hearing loss was initially evident, resolving both subjectively and on formal audiological evaluation after administering thyroxine. Central pathology affecting the eighth cranial nerve, as opposed to a conductive or mixed component is the likely culprit.

Alpha-1-Blockers Are Useful in the Symptomatic Management of Obstructive Salivary Gland Diseases


Presented at AAO-HNSF
By Cheryl Lathrop
BOSTON -- October 3, 2010 -- Alfuzosin was well tolerated and improved symptoms
related to stenosis or lithiasis of the parotid and submandibular glands in a
majority of patients, according to results of a study presented here on
September 27 at the American Academy of Otolaryngology-Head and Neck Surgery
Foundation (AAO-HNSF) Annual Meeting 2010.
Philippe Katz, MD, Institut d’Explorations Fonctionnelles des Glandes
Salivaires, Paris, France, and colleagues retrospectively analysed the files of
patients treated with alfuzosin between January 2005 to January 2008 for
submandibular or parotid ductal stenosis, allergic pseudo-parotitis, or remnant
sialolithiasis after extracorporeal lithotripsy to the parotid or submandibular
glands.
At the first 3-month follow-up visit, an ultrasound was performed and a
subjective evaluation of salivary symptoms was obtained by questionnaire.
Patients then had an ultrasound every 3 months. If treatment was well
tolerated, it was continued for 2 years. Treatment was discontinued if side
effects occurred (patients were questioned about side effects at every 3-month
visit) or if salivary symptoms completely resolved.
Of the 352 patients (282 male, 70 female) 69 had ductal stenosis, 89 had
allergic pseudo-parotitis, 112 had residual parotid lithiasis, and 82 had
residual submandibular lithiasis.
At the first 3-month visit, 80% of the patients with stenosis, 79% of the
patients with allergic pseudo-parotitis, and 67% of the patients with residual
parotid lithiasis reported “very much improved” or “completely resolved.”
However, only 42% of the patients with residual submandibular lithiasis
reported a large improvement (significantly lower than for the other 3
diseases), which the researchers found surprising.
The average follow-up was 33 months. No male/female difference was noted for
safety or efficacy.
Alfuzosin was well tolerated with only 12 (3.4%) patients reporting adverse
effects. Ten patients discontinued the treatment within the first 3 months. The
low complication rate and high efficacy rate imply that alpha-blockers may be
useful in the symptomatic management of obstructive salivary gland diseases.
“A randomised controlled study is warranted to confirm the efficacy of this
approach as compared to conventional treatments,” the researchers noted.
[Presentation title: Alpha-1-Blockers for Obstructive Salivary Gland
Diseases. Abstract 194]

Friday, October 8, 2010

Ear canal cholesteatoma: meta-analysis of clinical characteristics with update on classification, staging and treatment

Current Opinion in Otolaryngology & Head and Neck Surgery (Aug 2010)


Dubach P, Mantokoudis G, Caversaccio M; 

PURPOSE OF REVIEW: We present an update on clinical evaluation, staging, classification and treatment of canal cholesteatoma, including a meta-analysis of clinical data of the last 30 years. 

RECENT FINDINGS: Ear canal cholesteatoma is frequently associated secondarily to other canal pathologies. The cause for the rare idiopathic form of the disease remains enigmatic. Epidemiologic and experimental studies of its pathogenesis have increased; however, the main explanatory theory of a deficient migratory capacity of the canal epithelium affected has been falsified only recently. Therefore, the debate on the pathogenesis has gained additional impetus and more data is needed. 

SUMMARY: Canal cholesteatoma is a rarity in otologic pathology, often leading to misdiagnosis as external otitis or otomycosis by physicians unfamiliar with the disease. It presents typically with otorrhea, focal erosion and keratin accumulation in the osseous ear canal and has to be distinguished from keratosis obturans, which leads to otalgia and bilateral conductive hearing loss by ceruminal plugs, with circumferential distention of the ear canal. Treatment by canaloplasty is curative and highly successful. Alternative conservative treatment is feasible, however, requiring long-term follow up, with often painful cleaning of the lesion.

Friday, October 1, 2010

Bilateral symptomatic petrous apex effusion


Volume 62, Number 2186-188DOI: 10.1007/s12070-010-0034-4

Muzeyyen YildirimSenem SenturkEbru GuzelAslan Guzel and Ismail Topcu

Abstract

Petrous apex effusions can present with aural fullness, hearing loss and dizziness. Although they can be followed-up when asymptomatic, clinical management of symptomatic patients is controversial. In this study, we present clinical and radiological findings of a 24-year-old patient with bilateral petrous apex effusion. She had been complaining of bilateral aural fullness and dizziness for 2 years. Radiological examinations revealed bilateral petrous apex effusion. After medical treatment, her symptoms gradually disappeared. In all previous published studies, unilateral petrous apex effusions were reported. To our best knowledge, this is the first patient with trapped fluid in bilateral petrous apex.