Titanium implants in middle ear surgery were introduced in the late 90s and are now frequently used in middle ear surgery. However, long-term studies of patient outcome are few and have only been published in subgroups of patients. We report the long-term effect of titanium middle ear implants for ossicular reconstruction in chronic ear disease investigated in a Norwegian tertiary otological referral centre. Retrospective chart reviews were performed for procedures involving 76 titanium implants between 2000 and 2007. All patients who underwent surgery using the Kurz Vario titanium implant were included in the study. Audiological parameters using four frequencies, 0.5, 1, 2, and 3 kHz, according to AAO-HNS guidelines, was assessed pre and postoperatively. Otosurgical procedures, complications, revisions, and extrusion rates were analyzed. The study had no dropouts. The partial ossicular replacement prosthesis (PORP) was used in 44 procedures and the total ossicular replacement prosthesis (TORP) in 32 procedures, respectively. Mean follow-up was 5.2 years (62 months). The ossiculoplasties were performed as staging procedures or in combination with other chronic ear surgery. The same surgeon performed all the procedures. A postoperative air-bone gap of ≤ 20 dB was obtained in 74 % of the patients, 82 % for the Bell (PORP) prosthesis, and 63 % for the Arial (TORP) prosthesis. The extrusion rate was 5 %. We conclude that titanium ossicular implants give stable and excellent long-term hearing results.

BackgroundHearing loss (HL) is a well-known feature of Fabry disease (FD). Its presence and characteristics have mainly been studied in adult patients, while only limited data are available on the presence and degree of HL in children with FD. This prompted us to study hearing sensitivity in pediatric FD patients.MethodsAll available audiograms of the Dutch and Norwegian children with FD were retrospectively collected. First, hearing sensitivity was determined by studying hearing thresholds at low, high, and ultra-high frequencies in children with FD and comparing them to zero dB HL, i.e., healthy children. In addition, the presence and type of slight/mild HL (defined as hearing thresholds at low frequencies of 25–40 dB HL) and moderate to severe HL (hearing thresholds >40 dB HL) at first visit were analyzed. If available, follow-up data were used to estimate the natural course of hearing sensitivity and HL in children with FD.ResultsOne-hundred-thirteen audiograms of 47 children with FD (20 boys, median age at first audiogram 12.0 (range 5.1–18.0) years) were analyzed. At baseline, slight/mild or moderate to severe HL was present in three children (6.4%, 2 boys). Follow-up measurements showed that three additional children developed HL before the age of 18. Of these six children, five had sensorineural HL, most likely caused by FD. Compared to healthy children (zero dB HL), FD children showed increased hearing thresholds at all frequencies (p < 0.01), which was most prominent at ultra-high frequencies (>8 kHz). Hearing sensitivity at these ultra-high frequencies deteriorated in a period of 5 years of follow-up.ConclusionA minority of children with FD show slight/mild or moderate to severe HL, but their hearing thresholds are poorer than the reference values for normal-hearing children. Clinical trials in FD children should demonstrate whether HL can be prevented or reversed by early treatment and should specifically study ultra-high frequencies.Electronic supplementary materialThe online version of this article (doi:10.1007/s10545-017-0051-5) contains supplementary material, which is available to authorized users.

The most common technique in sound restoration of the middle ear is prosthetic surgery. Hypoventilation of the middle ear may cause adhesive otitis or atelectasis resulting in a higher risk of prosthetic extrusion rate and recurrence of the underlying cholesteatoma. We report long-term results using an island of tragal cartilage as an autologous PORP in selected patients with poor middle ear ventilation. Retrospective chart reviews were performed for procedures involving 52 patients between year 2000 and 2009. All patients that underwent surgery using tragal cartilage interposed between the suprastructure of the stapes and the tympanic membrane were included in this study. Audiological parameters using four frequencies, 0.5, 1, 2 and 3 kHz, according to AAO-HNS guidelines, were assessed pre-and postoperatively. The hearing results on different PTA frequencies were also investigated. We report long-term follow-up of patients with hypoventilated middle ear with a success rate of 71% (ABG <20%). With regards to the ABG, the low frequency component (5 and 1 kHz) showed a significantly (p < 0.05) larger improvement of mean values after surgery as compared to the high-frequency component (2 and 3 kHz). Cartilage island PORP on stapes is a stable and efficient method for selected patients with chronic middle ear disease.

Øre-nese-halsavdelingen Haukeland universitetssjukehus og Klinisk institu 1 Universitetet i Bergen Han har bidra med idé, revisjon av manus og godkjenning av innsendte versjon av manus. Frederik Kragerud Goplen er ph.d., overlege og førsteamanuensis II. Forfa eren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter.Øre-nese-halsavdelingen Haukeland universitetssjukehus Hun har bidra med idé, revisjon av manus og godkjenning av innsendte versjon av manus. Jeane e Hess-Erga er overlege og seksjonsleder. Forfa eren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter.Øre-nese-halsavdelingen Oslo universitetssykehus, Rikshospitalet Han har bidra med idé, revisjon av manus og godkjenning av innsendte versjon av manus. Leif Runar Opheim er overlege og seksjonsleder. Forfa eren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter.Øre-nese-halsavdelingen Akershus universitetssykehus og Klinikk for kirurgiske fag Universitetet i Oslo Han har bidra med idé, revisjon av manus og godkjenning av innsendte versjon av manus. Juha Tapio Silvola er ph.d., overlege, seksjonsleder og professor II. Forfa eren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter.Øre-nese-halsavdelingen St. Olavs hospital Hun har bidra med idé, revisjon av manus og godkjenning av innsendte versjon av manus. Brit Kari Stene er overlege. Forfa eren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter.

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