E. Ferekidis

JUVENILE RECURRENT RESPIRATORY PAPILLOMATOSIS: STILL A MYSTERY DISEASE WITH DIFFICULT MANAGEMENT

Juvenile recurrent respiratory papillomatosis (RRP) is the most common benign neoplastic disease of the larynx in children and adolescents and has a significant impact on patients and the health care system with a cost ranging from

Outcomes and efficacy of newborn hearing screening: strengths and weaknesses (success or failure?).

60,000 to

Simultaneous and Sequential Bilateral Sudden Sensorineural Hearing Loss: Are They Different from Unilateral Sudden Sensorineural Hearing Loss?

470,000 per patient. The aim of this paper is to review the current literature on RRP and summarize the recent advances. RRP is caused by human papillomavirus (HPV; mainly by types 6 and 11). Patients suffer from wart‐like growths in the aerodigestive tract. The course of the disease is unpredictable. Although spontaneous remission is possible, pulmonary spread and malignant transformation have been reported. Surgical excision, including new methods like the microdebrider, aims to secure an adequate airway and improve and maintain an acceptable voice. Repeated recurrences are common and thus overenthusiastic attempts to eradicate the disease may cause serious complications. When papillomas recur, old and new adjuvant methods may be tried. In addition, recent advances in immune system research may allow us to improve our treatment modalities and prevention strategies. A new vaccine is under trial to prevent HPV infection in women; the strongest risk factor for juvenile RRP is a maternal history of genital warts (transmitted from mother to child during delivery). Better understanding of the etiology of the disease and the knowledge of all available therapies is crucial for the best management of the affected patients.

Stapedotomy in osteogenesis imperfecta patients.

Objective: To assess the outcomes of neonatal hearing screening with regard to the final diagnosis in a very large number of newborns and investigate related strengths and weaknesses of the program.

Should We Use Ossicular Remnants in Ossicular Reconstruction following Cholesteatoma Removal

Aim: To compare bilateral (BSSHL) with unilateral (USSHL) sudden sensorineural hearing loss. Methods and Subjects: Two hundred and thirty-two patients with USSHL, 11 with simultaneous BSSHL and 7 with sequential BSSHL, who were older than 15 years had onset of hearing loss <30 days, no head injuries or history of acoustic trauma. All patients received the same treatment (prednisolone). Results: Hearing loss was more severe in simultaneous BSSHL in comparison to sequential BSSHL (p = 0.01) or USSHL (p = 0.03). Autoimmune diseases were far more common in simultaneous BSSHL (36% of patients) than USSHL. Positive antinuclear antibody was found in half of BSSHL patients and in only 8% of unilateral cases (p = 0.01). The frequency of hearing improvement was much lower in simultaneous BSSHL than in USSHL (p = 0.001). Complete or partial improvement was noted in 74% of unilateral cases versus 27% in simultaneous bilateral cases. Patients with sequential BSSHL improved in a similar way to unilateral cases. Conclusions: Simultaneous BSSHL, sequential BSSHL and USSHL may have a completely different profile and should not be managed as one disease. Hearing loss, underlying autoimmune diseases, antinuclear antibodies, and improvement/recovery of hearing loss vary in a degree that implies different pathophysiology and prognosis.

Lemierre's syndrome associated with consumption coagulopathy and acute renal failure: a case report

Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by osseous fragility, blue sclerae and hearing loss. In order to assess the impact of stapedotomy on improving hearing on OI, a retrospective, one-group, pre-test-post-test design was used to compare the pre-operative and post-operative audiograms of nine OI patients, treated with stapedotomy for their mixed hearing loss. Operative findings included fixation or thickening of the stapes footplate with normal superstructure configuration and hypervascularization of the promontory mucosa. Immediate post-operative results showed a significant improvement (p < 0.05) from 250-4000 Hz in air conduction and from 250-2000 Hz in bone conduction. A significant closure of the air-bone gap between 250-2000 Hz was also achieved (p < 0.05). The long-term results remained satisfactory with a mean threshold shift of 8 dB HL and an almost unchanged air-bone gap. These satisfactory results and the lack of complications make stapedotomy an appealing method for the management of OI-associated hearing loss.

Late Detection of Hearing Loss in a Case with Middle Ear Congenital Abnormality

The remnants of the acoustic ossicles have been used in ossicular reconstruction during mastoid surgery for many decades. The present study assessed the status of the acoustic ossicles in 114 patients (57 with cholesteatoma and 57 without) during surgery for chronic otitis media using the operating microscope. In 52 cases, the ossicles (malleus and/or incus) were assessed using both the surgical and scanning electron microscope in order to reveal any erosions and compare the findings. From the 57 operated ears with cholesteatoma, 45 (79%) had ossicular erosion whereas 12 (21%) did not. In the group of 57 operated ears with chronic otitis media without cholesteatoma, 33 (58%) had ossicular erosion whereas 24 (42%) did not. This difference was statistically significant (p = 0.02). With regard to the 52 operated cases who were studied with both microscopes, in the cholesteatoma patients the surgical microscope was not able to reveal any ossicular erosions in 39% of the cases, whereas the scanning electron microscope revealed moderate or severe erosions in the same ears. This suggests that the operating microscope is not reliable enough to determine if ossicular remnants can be used in ossicular reconstruction following cholesteatoma surgery. There is a considerable risk that epithelia or other cholesteatoma particles remain in the areas of erosions that cannot be seen with the operating microscope. The use of such ossicular remnants may lead to cholesteatoma recurrence and failures in mastoid surgery. Therefore, autoclaving or alternative prosthesis may be considered in such cases.

Laryngeal tuberculosis at the end of the 20th century.

BACKGROUND Acute tonsillitis or pharyngitis may lead to suppurative thrombophlebitis of the internal jugular vein. This complication, also known as Lemierres syndrome, remains, even nowadays, life threatening, due to dissemination of septic thromboemboli to various organs. Respiratory deficiency and renal impairment are often reported in patients suffering from Lemierres syndrome. CASE REPORT The unusual clinical manifestation of this case involves severe acute renal and respiratory deficiency in addition to microangiopathic consumption coagulopathy in a young patient treated with macrolides five days after the onset of acute tonsillitis. CONCLUSION The usual causative pathogen, namely Fusobacterium necrophorum, shows a varying sensitivity to macrolides. As a result, the syndrome may present itself in a variety of clinical forms even in patients under treatment with macrolides. A high index of suspicion is therefore crucial for in time prevention of potentially life threatening complications.

Subject Index Vol. 12, 2002–2003

The present paper describes a rare case of a unilateral congenital ossicular abnormality that presented in adulthood. A unilateral conductive hearing loss was revealed during a routine examination for a professional driving license. Differential diagnosis included otosclerosis and ossicular anomalies. Our patient, a 40-year-old man, had no history of otitis media or trauma to the head and he was not aware of his hearing loss. Exploratory tympanotomy revealed absence of the long process of the incus and of the stapes. A remnant of the underdeveloped stapes footplate was removed and reconstruction of the defect using a 5.0-mm malleus-oval window prosthesis had an excellent result with complete restoration of the air-bone gap. The patient experienced binaural hearing with great excitement.