Localized erosive pustular dermatosis of the scalp at the site of a cochlear implant: successful treatment with topical tacrolimus.

Erosive pustular dermatosis of the scalp (EPDS) is a rare form of nonmicrobial pustulosis mainly occurring in elderly patients with long-term sun damage to the skin. Clinically, it is characterized by pustular lesions that progressively merge into erosive and crusted areas over the scalp. The histology of EPDS is nonspecific, and its pathophysiology remains undetermined, with various types of local trauma possibly acting as the triggering factor. We describe a 24-year-old woman who developed EPDS after cochlear implant surgery for profound sensorineural hearing loss. We speculate that either the cutaneous surgery during cochlear implantation or the skin inflammation that commonly occurs near the magnet might have triggered the disorder. It is of note that the patient's skin lesions healed completely after treatment with topical tacrolimus, a relatively novel immunosuppressive molecule. Thus, topical tacrolimus may be indicated as a therapeutic alternative to the widely used steroids for this disease, mainly to avoid steroid-related cutaneous atrophy.

Open-set speech perception in adult cochlear implant users with ossified cochleae.

This study describes open-set speech recognition in cochlear implant subjects with ossified cochleae and compares it to a control group with open cochleae. Twenty-one postlingually deafened adults with a Med-El Combi 40/40+GB split- electrode implant were matched to patients using a Med-El cochlear implant with a standard electrode. Speech recognition was assessed over an 18-month period. Split- electrode patients improved significantly over time, but their scores were significantly lower and increased significantly slower than those of controls. Of 14 patients with a duration of deafness less than 20 years, average sentence test scores were 50%, and average monosyllabic word test scores were 31%. This study provides evidence that cochlear implantation is beneficial to patients with ossified cochleae, but early implantation is advisable.

Myringoplasty in children: anatomical and functional results.

To assess the results of myringoplasty in children and determine the factors influencing post-operative results a retrospective study of the anatomical and functional results of 41 myringoplasties in children was performed, considering only the cases of uncomplicated perforation that did not require ossiculoplasty or mastoidectomy. The overall success rate was 80.5 per cent after a mean follow-up of 39 months. The mean post-operative air conduction threshold significantly improved in the successful cases with a mean audiological improvement of 11 dB (p<0.05). No post-operative sensorineural hearing loss was observed. There was a significant statistical association between the presence of a dry ear at the time of surgery and good surgical results (p<0.01). Surgical outcome was not affected by the patient's age, the site and size of the perforation, previous adenoidectomy, surgical technique (overlay vs underlay), or the status of the contralateral ear. Our findings suggest that myringoplasty is a valid procedure in the paediatric population that gives good anatomical and functional results. The status of the middle ear (i.e. the presence of a dry ear), significantly improves surgical outcome; and so careful inflammatory changes in the middle-ear mucosa should be evaluated and medical treatment considered before surgery.

Incidence of genetic factors in the causation of deafness in childhood.

Routine investigations of 1568 children with severe-profound hearing loss, during a 15 years' period, revealed the following classification: Hereditary Deafness 25%, Acquired Deafness 43% (prenatally 11%, perinatally 16%, postnatally 16%), Unknown Deafness 32%. This group of unknown causes is usually large in any surgery of deafness and in many of these children deafness is believed to have a genetic basis. In the light of this premise, we performed more careful genetic and clinical investigation in 268 children coming under observation in the 3 last years. Moreover, we examined a group of 44 asymptomatic parental pairs of these children to determine if any were carriers of genes for deafness. The criteria used for identification were: pure tone audiometry, speech discrimination score, threshold of the acoustic reflex, Lüscher test, loudness discomfort level for 1-2-4 kHz, continuous Bekesy audiometry. Children follow-up enabled us to place 14 cases in the range of hereditary syndromes. Therefore, the group of unknown deafness decreased from 32% to 27%. The audiological investigation, performed on the parental pairs, pointed out an abnormal auditory function in 30.6% of subjects, who for these reasons should be considered as heterozygous carriers.

Some observations concerning 200 stapedectomies: fifteen years postoperatively.

In this study, a statistical evaluation of the hearing improvement, the incidence of postoperative tinnitus and vertigo, and the permanency of the surgically restored hearing of 120 women and 80 men operated on for otosclerosis between 1970 and 1971 was undertaken. The standardized operating procedure in all of these cases involved the use of a Gelfoam and wire prosthesis (Schuknecht 3). All patients underwent normal audiological examinations, including determination of bone and air conduction values. Pure tone thresholds were determined by averaging the values for 500, 1,000, 2,000 and 4,000 Hz. The incidence (10%) of severe sensorineural loss in regard to age, preoperative bone conduction, and oval window pathology was examined. Severe sensorineural losses had occurred immediately postoperatively in 14 of the ears. In the remaining ears, losses were gradual in the course of 15 years. Hearing thresholds in ears not subjected to operation were also determined.

[Follow-up and prognosis of congenital hypothyroidism]. / Follow-up e prognosi dell'ipotiroidismo congenito.

Neonatal screening of congenital hypothyroidism has been recently extended to the most of North America, Australia, Europe, and to several Italian areas. Before screening programs, several Authors reported neurological defects and behavioral disturbances also in patients whose treatment has been precocious, thus stressing the importance of an antenatal thyroidal defect. We have therefore setted up a follow-up program to evaluate the prevalence and to treat such disturbances in hypothyroid children. In this report we describe the program, present the most significant preliminary data and discuss the prognosis of hypothyroid patients detected by screening programs.