The objective of this review is to analyze aspects of congenital malformation of the ear in relation to cochlear implantation in children. Having briefly described the in utero development of the ear and the classification of types of external, middle and inner ear malformation, five practical aspects of these malformations are discussed. It seems likely that the combination of bilateral profound sensorineural deafness with bilateral microtia severe enough to make a surgical approach to the cochlea difficult will be extremely uncommon. No such cases have been reported, although Klippel-Feil deformity seems the syndrome most likely to produce this set of circumstances.

Abnormalities in the intratympanic course of the facial nerve have been associated with cochlear malformation, emphasizing the benefit of intra-operative facial nerve monitoring, and a technique suggested for safely avoiding an abnormally placed nerve. Fistulae of cerebrospinal fluid (CSF) and perilymph can complicate surgery and are relatively common in common cavity and Mondini malformations. Strategies for facilitating surgery in the presence of ‘gushers’, for measuring the pressure of a gusher and for placement of the cochlear implant electrode array are reviewed, with reports of fluctuating levels of electric current when implants lie in dysplastic cochleas.

The relationship of implant performance to VIIIth nerve tissue in malformed cochleas is discussed, with a description of the histological findings in a common cavity cochlea. Techniques for identifying the absence of the cochlear nerve are reviewed. Stimulation of the facial nerve by cochlear implants has been described in cases of congenital malformation of the labyrinth but is relatively uncommon. Case reports of the benefit received by implanted children with congenital cochlear malformation have appeared since 1988. Most cases reported have not yet been followed for long enough to establish a clear picture of the outcome following cochlear implantationin such children; no centre has yet built up a large series of cases, but there have been two multicentre postal surveys. It seems likely that in cochlear malformation the range of potential outcomes in terms of hearing threshold and the development of speech perception and production will be similar to the range found in implanted children without cochlear dysplasia. However there is, as yet, no clear picture of the mean level of performance within this range.

In November 1943, one of Barbara Hepworth’s daughters (Sarah, one of triplets) developed osteomyelitis of the thigh. This necessitated operation and subsequent treatment at the Princess Elizabeth Orthopaedic Centre in Exeter by Mr. Norman Capener. Artist and surgeon subsequently became friends and she encouraged his activities as a ‘weekend sculptor’. At his invitation she came to the hospital in November 1947 watching him in out-patients but more particularly in the operating theatre; other visits to London and Exeter followed. These studies of orthopaedic procedures form the great majority of Hepworth’s sixty-plus hospital drawings/paintings but there is also a small group of six paintings involving an operation on the ear made in April and May 1948. The link between these two and the introduction to the ear surgeon, Mr Garnett Passe, is unclear.

Five of the paintings of the ear operation are still in circulation but there is also a sketchbook containing no fewer than 28 drawings. A selection of the sketches together with the fenestration series of paintings will be discussed and also the relationship with the surgeon, Mr Garnett Passe.